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    Oncology oite-review-2012 Oncology oite-review-2012 Presentation Transcript

    • Orthopaedic Oncology OITE Review 2012 8.29.2012 Ruth Delaney, MDSpecial thanks to Matteos, Boulton, Kujo, First Aid for the Boards Those who have come before us
    • Introduction• History: age, sex, duration of sx, presence and quality of pain, history of trauma, weight loss, smoking history and history of prior malignancy – Red flags: pain that extends beyond expected duration, night pain, T- spine pain – Beware the history of trauma
    • Radiographic Differential DiagnosisBone Forming Tumors Cartilage Forming Tumors The RestOsteoid Osteoma Osteochondroma InfectionOsteoblastoma Chondromyxoid fibroma MetastasesOsteosarcoma Chondroblastoma Round cell tumorsBlastic Metastases Enchondroma Fibrous DysplasiaPagets Disease Chondrosarcoma Non-Ossifying Fibroma(Fibrous Dysplasia) Simple Bone Cyst Aneurysmal Bone Cyst Histiocytosis Giant cell tumor Metabolic condition PINK BLUE
    • Bone-Producing Tumors• Osteoid Osteoma• Osteoblastoma• Enostosis (bone island)• Osteosarcoma
    • Bone Forming Tumors • Possibilities: fracture callus, myositis ossificans, fibrous dysplasia, osteosarcoma, osteoblastoma, osteoid osteoma • WOVEN bone & spindle cell stroma – Bone differentiates reactive from neoplastic – Stromal cells differentiate benign from malignant • Woven bone: reactive (OB rimming) vs neoplastic (no OB rimming) • Malignant stroma: hypercellularity, atypia, pleomorphism, high mitotic rate, etc.
    • Osteoid Osteoma• Most common first two decades• Classic pattern of constant pain relieved by ASA/NSAIDs• Proximal femur is the most common location followed by the tibia, posterior elements of the spine, and the humerus• Osteoid Osteoma is found in the diaphysis or the metaphysis of the proximal end of the bone more often than the distal end• Small lytic nidus with target appearance• Four diagnostic features include (1) a sharp round or oval lesion that is (2) less than 2 cm in diameter, (3) has a homogeneous dense center and (4) a 1-2 mm peripheral • CT scan as preferred method of radiolucent zone evaluation
    • Osteoid Osteoma
    • Osteoid Osteoma• Histology: Osteoid trabeculae in loose, vascular, stromal connective tissue• Rx: – NSAIDs x 2 yrs (50% successful) – CT-guided radiofrequency ablation (95% successful)
    • Osteoid Osteoma• Affects young (<30 yo old), lower extremity, pain gets better with NSAIDS• Imaging shows a central nidus with reactive bone, hot on bone scan, always <1-1.5cm.• Treatment: RF ablation• Histology: woven bone, sharp border• *most common benign bone tumor of carpal bones
    • Osteoblastoma • ~80% of these tumors occur in patients younger than 30 years • ~40% located in the spine-- usually involve the posterior elements, and 17% of spinal osteoblastomas in the sacrum • Osteoblastoma of the long tubular bones (LE>>UE) is often diaphyseal • On x-ray--appear as a radio- lucent defect with a central density due to ossification. The lesion is well circumscribed and may have a surrounding sclerosis • Classic: calcified lesion in posterior elements of the spine
    • Osteoblastoma • Histology: Osteoblastic rimming of trabeculae • Rx: excision with at least a Marginal Margin because recurrence rate 20% (40%+ w/curettage)
    • Osteoid Osteoma vs. Osteoblastoma• Common • Uncommon• Usually < 1 cm • Usually > 2 cm• Regular tissue pattern • Irregular tissue• Pain pattern constant/nocturnal • Pain sporadic• Pain often relieved with NSAIDs • Pain not relieved with• Axial involvement NSAIDs uncommon • Axial involvement common
    • Bone Island or Enostosis• Focus of mature cortical bone within the cancellous bone• Cold on bone scan
    • Osteosarcoma• Osteosarcoma is very rare in young children• Incidence increases steadily with age, increasing more dramatically in adolescence, corresponding with the growth spurt• Typically occurs in first 3 decades with second peak beyond sixth decade
    • Osteosarcoma• most commonly occurs in the extremities of long bones near metaphyseal growth plates• most common sites are the femur (42%, 75% of which are distal femur), tibia (19%, 80% of which are proximal tibia), and humerus (10%, 90% of which are proximal humerus)• other significant locations are the skull and jaw (8%) and pelvis (8%)
    • Osteosarcoma• most commonly occurs in the extremities of long bones near metaphyseal growth plate• OCCURS AT MOST METABOLICALLY ACTIVE SITES• The most common sites are the femur (41%, 75% of which are distal femur), tibia (19%, 80% of which are proximal tibia), and humerus (10%, 90% of which are proximal humerus).• Other significant locations are the skull and jaw (8%) and Most in the Distal Femur!!! pelvis (8%) Second Most in Proximal Tibia!!!
    • Osteosarcoma • most common presenting symptom is pain, particularly pain with activity • lesions can be purely osteolytic (~30% of cases), purely osteoblastic (~45% of cases), or a mixture of both • On xray, permeative metaphyseal lesions with soft tissue extension and new bone formation • Periosteal reaction is common and frequently takes on a ―sunburst‖ appearance • MRI of the primary lesion is the best method to assess the extent of intramedullary disease as well as associated soft tissue masses and skip lesions
    • Osteosarcoma • Histology: spindle– shaped tumor cells that produce osteoid • Rx:Neo chemorestage resection Maintenance chemo = 60-70% long term survival with localized OSA • Surgery alone = 10-20% pt survival • Increased risk OSA: missing Rb gene (Ch13), Paget’s, prior XRT
    • Osteosarcoma Sub-types • Conventional (~90%)- usually begins in medullary canal • Parosteal (~5%)-low grade ―lobulated‖ attachment to cortex/75% at posterior aspect of distal femur • Telengiectatic (~4%)-purely lytic, ―bag of blood‖, confused with ABC but has spindle cell • Periosteal (2%)-juxtacortical, diaphyseal, ―sunburst,‖ creates ―crater‖ • Others: well-diff, IM, small cell, multicentric
    • Osteosarcoma Sub-Types• High- grade intramedullary• Telangiectatic• Parosteal• Periosteal
    • High-grade intramedullary• Most Common type of OS• 75% present as IIB, and the other 25% present as mets (lung)• 50% Occur around the knee• *image entire bone to look for skip mets (equivalent to mets).• Hot on Bone scan, Histology is pink osteoid.• Tx-Chemo, surgery, chemo• Percentage of tumor necrosis in response to chemo is main prognostic factor.
    • Telangiectatic Osteosarcoma• Looks similar to ABC – Watch out• Lytic with no mineralization• * if located on ulnar side of DR= OS• * if located on radial side of DR = GCT
    • Parosteal Osteosarcoma• Low grade surface lesion- ―stuck on bone‖• 80% distal posterior femur, painless mass• Often confused with Fibrous dysplasia by pathologist• Bland spindle cells around the bone• Treatment- wide surgical excision- NO CHEMO.
    • Periosteal Osteosarcoma• Rare, diaphyseal location, second decade, sunburst pattern , chondroblastic• Treatment: chemo, surgery, chemo• Prognosis is worse than parosteal but not as bad as high-grade intramedullary.
    • Summary – Osteoblastoma & Osteosarcoma
    • Osteoblastoma• Big brother to osteoid osteoma, pain NOT relieved by NSAIDS.• 2-6cm in size.• Posterior elements of spine most common location.• Histology: woven bone with distinct borders.
    • Osteosarcoma• Most common primary sarcoma of bone• Affects young patients• Associated with retinoblastoma• Most commonly presents in the 2nd and 3rd decade of life (also in older adults with Pagets)• Best predictor of survival is the stage of the disease.• Most common presentation is Stage IIB
    • Other Bone-Forming Conditions
    • Other Bone-Forming Conditions• Blastic Metastases: permeative lesion with infrequent soft tissue extension – 30/60/90 rule renal/breast/prostate• Paget’s: early lyticlate blastic (coarse trabeculae and bony enlargement—mosaic pattern) – OITE Pearls: • Assoc w/Paramyxovirus • increased cranial diameter (hats don’t fit) • deafness (2/2 nerve compression)
    • Fibrous Dysplasia• Peak age of diagnosis is 5 - 20 years – Two thirds of patients with polyostostic disease are symptomatic before the age of 10 – With monostotic disease, patients as old as 20 - 30 years are asymptomatic• ~70-80% are monostotic – This form most frequently occurs in the rib (28%), femur (23%), tibia or craniofacial bones (10-25%), humerus, and vertebrae, in decreasing order of frequency• ~20-30% of fibrous dysplasias are polyostotic – This form more frequently involves the skull and facial bones, pelvis, spine, and shoulder girdle – The sites of involvement are the femur (91%), tibia (81%), pelvis (78%), ribs, skull and facial bones (50%), upper extremities, lumbar spine, clavicle, and cervical spine, in decreasing order of frequency
    • Fibrous Dysplasia • Xrays: lucent lesion in the diaphysis or metaphysis, with endosteal scalloping and with or without bone expansion and the absence of periosteal reaction – Usually, the matrix is smooth and relatively homogeneous; classically, described as ―ground-glass‖ appearance – Classic ―Shepherd’s crook‖ deformity
    • Fibrous Dysplasia• Histology: irregular foci of woven bone arising from a cellular fibrous blue background – irregular trabeculae have been described as ―Chinese letters‖ or ―alphabet soup‖• Rx: Observation /benign/usually disappears – If >75% cortex or painfulcurettage/grafting – rigid, intramedullary fixation with the strongest possible device (a steel or titanium cephalomedullary nail) is the best method for treatment of proximal femoral lesions • McCune-Albright Syndrome: polyostotic, café au lait spots, precocious puberty, hyperthyroid Mutation is alpha subunit of a G protein
    • Myositis Ossificans• Juxtaposed to bone, and not connected to cortex• Can be confused with synovial cell sarcoma• Can be confused with parosteal OS• MO ossifies from periphery inward
    • Bone-Forming Lesions Histology Summary• Reactive lesion: woven bone with osteoblastic rimming and a benign spindle cell stroma (frx callus, myositis ossificans)• Benign or low-grade bone forming neoplasm: woven bone bone with no osteoblastic rimming and bland-appearing spindle cell sroma (osteoid osteoma, osteoblastoma, fibrous dysplasia)• Malignant bone tumor: woven bone with no osteoblastic rimming associated with malignant spindle cell stroma (osteosarcoma)
    • Bone-Forming Lesions OITE questions
    • OITE QUESTION85. A 15 yo cross country runner reports shin pain that is present during running, at rest, and at night. The pain is relieved by ibuprofen. Radiographs, a bone scan, and a CT scan are shown in Figures 32a through 32c. What is the most likely diagnosis?1. Cortical desmoid2. Osteoblastoma3. Stress fracture4. Enchondroma5. Osteoid osteoma
    • PINK = BONE
    • OITE QUESTION85. A 15 yo cross country runner reports shin pain that is present during running, at rest, and at night. The pain is relieved by ibuprofen. Radiographs, a bone scan, and a CT scan are shown in Figures 32a through 32c. What is the most likely diagnosis?1. Cortical desmoid2. Osteoblastoma3. Stress fracture4. Enchondroma5. Osteoid osteoma
    • OITE QUESTIONOsteosarcoma most commonly develops in which of the followinglocations?1- Pelvis2- Distal humerus3- Proximal tibia4- Proximal femur5- Proximal humerus
    • OITE QUESTION > > >OCCURS AT MOST METABOLICALLY ACTIVE SITES
    • OITE QUESTIONOsteosarcoma most commonly develops in which of the followinglocations?1- Pelvis2- Distal humerus3- Proximal tibia4- Proximal femur5- Proximal humerusPreferred Response: 3Recommended Reading(s):Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont,IL, American Academy of Orthopaedic Surgeons, 2002, pp 175-186.Mankin HJ, Hornicek FJ, Rosenberg AE, et al: Survival data for 648 patients withosteosarcoma treated at one institution. Clin Orthop Relat Res 2004;429:286-291
    • OITE QUESTIONA 9-year-old boy is seen for bilateral thigh pain. He has a history ofprecocious puberty. Examination reveals multiple café-au-lait spots. APpelvis and frog lateral hip radiographs are shown in Figures 21a and 21b.His condition is linked to an abnormality in1- G protein function.2- osteoclastic function.3- vitamin D metabolism.4- sulfate transporter gene.5- type I collagen formation.
    • OITE QUESTIONA 9-year-old boy is seen for bilateral thigh pain. He has a history ofprecocious puberty. Examination reveals multiple café-au-lait spots. APpelvis and frog lateral hip radiographs are shown in Figures 21a and 21b.His condition is linked to an abnormality in1- G protein function.2- osteoclastic function.3- vitamin D metabolism.4- sulfate transporter gene.5- type I collagen formation. Recommended Reading(s): DiCaprio MR, Enneking WF: Fibrous dysplasia: Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-1864. Parekh SG, Donthineni-Rao R, Ricchetti E, et al: Fibrous dysplasia. J Am Acad Orthop Surg 2004;12:305-313.
    • OITE QUESTION Which of the following diseases of bone (when nonmetastatic at diagnosis) carries the worst prognosis for 5-year survival)?• 1- Lymphoma• 2- Osteosarcoma• 3- Ewing’s sarcoma• 4- Paget’s sarcoma• 5- Conventional chondrosarcoma
    • OITE QUESTION Which of the following diseases of bone (when nonmetastatic at diagnosis) carries the worst prognosis for 5-year survival)?• 1- Lymphoma• 2- Osteosarcoma• 3- Ewing’s sarcoma• 4- Paget’s sarcoma• 5- Conventional chondrosarcoma
    • Cartilage-forming Lesions
    • Radiographic Differential DiagnosisBone Forming Tumors Cartilage Forming Tumors "Third List"Osteoid Osteoma Osteochondroma InfectionOsteoblastoma Chondromyxoid fibroma MetastasesOsteosarcoma Chondroblastoma Round cell tumorsBlastic Metastases Enchondroma Fibrous DysplasiaPagets Disease Chondrosarcoma Non-Ossifying Fibroma(Fibrous Dysplasia) Simple Bone Cyst Aneurysmal Bone Cyst Histiocytosis Giant cell tumor Metabolic condition
    • Cartilage-Producing Lesions• Enchondroma• Periosteal Chondroma• Osteochondroma• Multiple Hereditary Osteochondroma• Chondromyxoid Fibroma• Chondrosarcoma• Clear Cell Chondrosarcoma• Differentiated Chondrosarcoma
    • Cartilage Forming Tumors • Normal Cartilage: sparsely cellular, one cell per lacuna, one pyknotic nucleus per cell, well formed matrix • 3 tumor patterns: – Benign (enchondroma) merging into low grade which merges into intermediate then high grade – Chondroblastoma • Cobblestone chondroblasts and intervening chicken wire calcification – Chondromyxoid fibroma • Benign spindle cell lesion with some areas of immature cartilage
    • Enchondroma• Benign, metaphyseal, 60% are in the hand• Most common primary lesion in the hand• Imaging- stippled rings and arches, ―popcorn‖ calcifications, ―blue balls‖ of cartilage• Compared to ―smoke up the chimney‖ for bone infarct• Ollier’s disease- multiple lesions/ 30% malignant transformation• Mafucci disease- multiple bone lesions and hemangiomas- 100% malignant
    • Enchondroma • peak incidence 20-40 yo (Ollier’s 0-10 yo) • usually found in the short tubular bones of the hands and feet – most common primary tumor in the hand and is normally found in the diaphysis • NOT common in axial skeleton (if so, think chondrosarcoma) • Xrays: lucent area in medullary canal (may see endosteal scalloping) with variable mineralization (rings, stipples, arcs)‖popcorn‖
    • Enchondroma• Multiple enchondromas may occur in 3 distinct disorders: – Ollier disease: nonhereditary disorder characterized by multiple enchondromas with a predilection for unilateral distribution (30% develop low grade chondrosarcoma) – Maffucci syndrome: nonhereditary, less common than Ollier disease (100% develop chondrosarcoma) • +multiple hemangiomas • +other malignancies – follow with PET scan – Metachondromatosis: multiple enchondromas and osteochondromas • inherited by autosomal dominant transmission
    • Enchondroma
    • Enchondroma • Histology: mineralized hyaline cartilage – May see bone encased lobules separated by marrow • Rx: solitary painless enchondromas may be observed – Painful or worrisome lesions should be treated with biopsy followed by intralesional resection/curettage – Large defects can be filled with bone graft – All specimens must be analyzed carefully for malignancy.
    • Periosteal Chondroma• Rare• Occurs in 10-20 year olds• Presents under the periosteum• 50% at the proximal humerus
    • Osteochondroma• 35% of all benign lesions, very common• Does not grow in adulthood• Key feature is continuity with the medullary canal and with the cortex• Sessile, broad based and points away from the joint• Cartilage cap is less than 2cm, if bigger- be concerned for chondrosarcoma transformation 1%.
    • Osteochondroma• Histology: endochondral ossification on the basal surface of hyaline cartilage so it resembles a normal growth plate with rows of chondrocytes – cartilage more disorganized than normal, has binucleate chondrocytes in lacunae, and is covered with a thin layer of periosteum• Rx: Asymptomatic = Observation
    • MHE- Multiple Hereditary Exostosis• A subset of osteochondromas• Most frequent lesion to have secondary chondrosarcomas, especially if they grow after skeletal maturity.• Autosomal Dominant• EXT1, EXT2, EXT3
    • Osteochondroma• most common benign bone tumor• most commonly present in the second decade of life• grow until skeletal maturity and then stop once the growth plates fuse• most commonly found around the knee and the proximal humerus• Can be pedunculated or sessile• Hallmarks: Grow away from the physis and have medullary continuity• Secondary sarcomatous degeneration should be suspected if grows after puberty or has cartilage cap >3 cm in adulthood• OITE pearl: Osteochondromatosis (HMOCE - Hereditary Multiple Osteo- Cartilagenous Exostoses) – AD Ch8 (EXT1), Ch11 (EXT2) – risk of malignant transformation to chondrosarcoma may be 25- 30% compared to approximately 1% for a solitary osteochondromas
    • OITE QUESTIONPatients with multiple hereditary osteochondromas frequently haveloss of forearm rotation and ulnar shortening. What procedure ismost likely to improve forearm rotation in thesepatients?1- Tendon transfer2- Ulnar lengthening3- Radial head excision4- Distal ulnar resection5- Simple excision of the osteochondroma
    • OITE QUESTION
    • OITE QUESTION Patients with multiple hereditary osteochondromas frequently have loss of forearm rotation and ulnar shortening. What procedure is most likely to improve forearm rotation in these patients? 1- Tendon transfer 2- Ulnar lengthening 3- Radial head excision 4- Distal ulnar resection 5- Simple excision of the osteochondromaShin EK, Jones NF, Lawrence JF: Treatment of multiple hereditary osteochondromas of theforearm in children: A study of surgical procedures. J Bone Joint Surg Br 2006;88:255-260.Akita S, Murase T, Yonenobu K, et al: Long-term results of surgery for forearm deformitiesin patients with multiple cartilaginous exostoses. J Bone Joint Surg Am 2007;89:1993-1999.
    • Chondromyxoid Fibroma• Occurs in 20-30 year olds in proximal tibia or foot, metaphysis.• Eccentric, well-demarcated lesion, 25% recur• Treatment: Curretage and bone graft• Histology: stellate appearing cells, ―soap bubbles‖ or snot
    • Chondromyxoid fibroma • Extremely rare • Presents 2nd-3rd decades • found most often in the metaphysis around the knee in the proximal tibia, proximal fibula, or distal femur • Xray: eccentrically placed Iytic lesion with well defined margins in the metaphysis of the lower extremity • Histology: chondrocytes in myxoid matrix separated by fibrous bands • Rx: Extended curettage/Graft ~10% recurrence – En bloc excision as complete cure
    • Chondroblastoma• Epiphyseal lesion of the young about the knee, proximal humerus, and proximal femur• Painful-often abut the joint, and can cross the physis• 2-5% metastasis to the lungs• Calcaneus lesion = Codman’s tumor• Histology: Chicken-wire calcifications; cobblestone appearance• Treatment: Intralesional curretage and bone graft
    • Chondroblastoma • ~90% occur in those aged 5-25 years • Most common epiphyseal lesion in children behind infection • typically occurs in the epiphysis of a long bone • most common site is the lower extremity (72% of cases), and 50% of tumors occur around the knee – The femur is involved in 33% of cases; the humerus, in 20%; and the tibia, in 18% • symptoms are nonspecific and include joint pain, tenderness, swelling – Joint effusion occurs in approximately 30% of patients – Get an xray before you tap a joint!
    • Chondroblastoma • Xray: radiolucent with well defined margins – fine calcifications, either punctate or in rings, may be visible • Histology: densely packed polygonal Blue chondroblasts with ―Fried Egg‖ appearance and ―chicken wire‖ calcification • Rx: biopsy and curettage with +/- adjuvant liquid nitrogen or phenol, or a mechanical burr – ~20% recurrence rate • 2% mets to lungs • Classic: painful lucent lesion inBLUE = Cartilage the epiphysis of a child
    • Chondrosarcoma• Treatment is surgery!• Occurs in patients >50 yrs old• Most common location is the pelvis, ribs, and proximal femur• Axial and proximal skeletal lesions are more aggressive• Histologic grade correlates with mets:• Grade 1- 5%• Grade 2-20%• Grade 3- 60%• Dedifferentiated- almost 0% survival
    • Chondrosarcoma• 2nd most frequent primary malignant tumor of bone, representing approximately 25% of all primary osseous neoplasms• Usually occur in patients older than 40 years• THINK axial skeleton: most commonly involve the pelvic bones, femur, humerus, ribs, scapula, sternum, or spine – In tubular bones, the metaphysis is the most common site of origin• most common symptom at presentation is pain, often present for months and typically dull in character – may be worse at night – average duration of symptoms prior to presentation is 1-2 years
    • Chondrosarcoma• Xrays: fusiform, lucent defect with scalloping of the inner cortex and periosteal reaction – Extension into the soft tissue may be present as well as punctate or stippled calcification of the cartilage matrix• CT helpful in defining the integrity of the cortex and distribution of calcification• MRI invaluable in surgical planning as it demonstrates the intraosseus and soft tissue involvement of the tumor – also helpful in evaluating possible malignant degeneration of osteochondromas by allowing accurate measurements of the cartilage cap which should be less than 2 cm thick
    • Dedifferentiated Chondrosarcoma• Most Malignant cartilage lesion• 50% have a pathologic fracture due to tumor’s aggressive and very destructive nature.• Treatment- aggressive- chemo, surgery, chemo• 13% 5 year survival rate
    • Chondrosarcoma • Categorized by grade: – Grade 1 (Low grade)-low cellularity, abundant ECM, small dark nuclei (5% mets) – Grade 2 (Low grade)- increased cellularity [50% chondrosarc] (20% mets) – Grade 3 (High grade/ most aggressive)-high cellularity, mitotic figures, etc. (70% mets) • 75% are Low grade • Most classified as conventional, but other subgroups are clear cell, myxoid, mesenchymal, and dedifferentiated
    • Chondrosarcoma• Centralin Medullary cavity• Surface – Seen in 20-40 yo group most often in pelvis/prox femur – ―explosive‖ lesion of stippling that is NOT circumscribed• Dedifferentiated – mix of low grade chondrosarcoma and high grade spindle cell sarcoma where the spindle cells are no longer identifiable as having a cartilage origin • Mesenchymal – biphasic quality is on x-ray with predilection for the spine, ribs and jaw areas of endosteal and it presents in the third decade scalloping/cortical thickening rare variant with a bimorphic histologic contrasted with areas of cortical picture of low grade cartilaginous cells destruction and hypercellular small, uniform, and – Most malignant—5 yr surv 10% undifferentiated cells that resemble Ewings sarcoma • Clear cell
    • Chondrosarcoma • Clear cell – adult variant of chondroblastoma – found in the epiphysis of the femur and humerus • Classic—head of femur – Histology: clear cells with vacuolated cytoplasm • matrix has significantly calcified trabeculae and giant cells – rare, low-grade tumor with an improved prognosis over other chondrosarcomas • OITE pearls: Epiphyseal lesions = chondroblastoma (pedi), giant cell and clear cell (adult)
    • Chondrosarcoma• Treatment =Wide surgical excision• XRT and Chemo resistant• Biopsies must be planned with future tumor excision in mind• Patients with adequately resected low grade chondrosarcomas have an excellent survival rate• The survival of patients with high grade tumors depends on the location, size and stage of the tumor
    • Cartilage-Forming Lesions OITE Questions
    • OITE QUESTIONA 12-year-old patient has an epiphyseal lesion. What is themost likely diagnosis? 1- Giant cell tumor 2- Aneurysmal bone cyst 3- Eosinophilic granuloma 4- Dysplasia epiphysealis hemimelica 5- Chondroblastoma
    • OITE QUESTIONA 12-year-old patient has an epiphyseal lesion. What is themost likely diagnosis? 1- Giant cell tumor 2- Aneurysmal bone cyst 3- Eosinophilic granuloma 4- Dysplasia epiphysealis hemimelica 5- Chondroblastoma
    • OITE QUESTION• Which of the following conditions shown in these pictures has the highest rate of malignant change? 1. Ollier’s disease 2. Enchondromatosis 3. Maffucci’s syndrome 4. Multiple exostoses 5. Solitary osteochondroma
    • OITE QUESTION• Which of the following conditions shown in these pictures A-D has the highest rate of malignant change? 1. Ollier’s disease 2. Enchondromatosis 3. Maffucci’s syndrome 4. Multiple exostoses 5. Solitary osteochondroma
    • OITE QUESTION23. An 80-year-old woman reports the sudden development of pain in the left distal thigh. She denies any history of trauma. Figures 9a through 9d show radiographs, a bone scan, and a biopsy specimen. What is the likely diagnosis? 1- Enchondroma 2- Osteosarcoma 3- Dedifferentiated chondrosarcoma 4- Metastatic breast carcinoma 5- Paget’s disease
    • OITE 2006 – Ortho Diseases
    • OITE QUESTION23. An 80-year-old woman reports the sudden development of pain in the left distal thigh. She denies any history of trauma. Figures 9a through 9d show radiographs, a bone scan, and a biopsy specimen. What is the likely diagnosis? 1- Enchondroma 2- Osteosarcoma 3- Dedifferentiated chondrosarcoma 4- Metastatic breast carcinoma 5- Paget’s disease
    • Radiographic Differential DiagnosisBone Forming Tumors Cartilage Forming Tumors "Third List"Osteoid Osteoma Osteochondroma InfectionOsteoblastoma Chondromyxoid fibroma MetastasesOsteosarcoma Chondroblastoma Round cell tumorsBlastic Metastases Enchondroma Fibrous DysplasiaPagets Disease Chondrosarcoma Non-Ossifying Fibroma(Fibrous Dysplasia) Simple Bone Cyst Aneurysmal Bone Cyst Histiocytosis Giant cell tumor Metabolic condition
    • ―Third List‖ Tumors• Fibrous Dysplasia (COVERED IN BONE PRODUCING LESIONS)• Non-ossifying fibroma• Osteofibrous dysplasia• Aneurysmal Bone cyst• Unicameral Bone cyst• Myeloma• Lymphoma• Ewing’s Sarcoma• Neuroblastoma• Histiocytosis• Giant Cell Tumor
    • Fibrous Dysplasia• Developmental abnormality that can be mono- or polyostotic• Café au lait spots- Coast of Maine (more rugged)• Coast of California (smoother) — neurofibromatosis• Associated with Gs-alpha Protein• Common location is proximal femur- shepard’s crook.• X-ray has ground glass appearance
    • Fibrous Dysplasia • Peak age of diagnosis is 5 to 20 years • sites of involvement are the femur (91%), tibia (81%), pelvis (78%), ribs, skull and facial bones (50%), upper extremities, lumbar spine, clavicle, and cervical spine, in decreasing order of frequency • Xrays: lucent lesion in the diaphysis or metaphysis, with endosteal scalloping and with or without bone expansion and the absence of periosteal reaction – ―ground glass‖ appearance • Rx: Observation b/benign/usually disappears – If >75% cortex or painfulcurettage/grafting
    • LEARN FROM THE MISTAKES OF OTHERS IN FIBROUS DYSPLASIA• Do NOT use autograft• Do NOT use cancellous allograft• Do NOT try to remove all of the disease• Do NOT allow the deformity to be come severe• Do NOT use plates and screws if possible• Do NOT expect a single operation to solve the problem Stanton, Robert P. J Bone & Mineral Res, 2006; 21:p105-109.
    • Osteofibrous Dysplasia• A.k.a ossifying fibroma• most common site in adults is the mandible• most common site in children is the tibia, followed by other long bones• occurs during the first decade of life and presents clinically as a painless, enlarging mass• XRAY: multiple eccentric, well-circumscribed lucent lesions in anterior cortex of tibia• Histology: irregular spicules of trabecular bone lined by osteoblasts in fibroblastic background – stains positive for cytokeratin
    • Osteofibrous Dysplasia• Treatment – Older patients : • conservative management • Curettage – Pts w/open growth plates • observe • if bowing of the tibia becomes an issue, bracing is recommended
    • Fibrous Dysplasia vs Osteofibrous Dysplasia Fibrous Dysplasia Osteofibrous Dysplasia – less well circumscribed bone surrounded by osteoblasts
    • Non-ossifying fibroma (fibrous cortical defect) • Xrays: eccentric, multi- loculated sub-cortical lesions with a central lucency and a scalloped sclerotic margin • Histology: highly cellular (including giant cells & lipophages) with storiform fibrogenic areas • Rx: observation (most regress spontaneously) – only definite indication to treat is a pathologic fracture • *Pearl - Jaffe-Campanacci syndrome: multiple NOF, cafe- au-lait spots, mental retardation, hypogonadism, ocular/cardio abnormalities.
    • Non-ossifying fibroma (fibrous cortical defect)• found mostly in children with 75% occurring in the second decade• non-neoplastic process that occurs in the juxta-epiphyseal region of the long bones – ~90% involve the tubular long bones – Common sites include the femur (most commonly the distal femoral metaphysis 38%), the proximal and distal tibia (43%) – most lesions occur around the knee• Classic scenario: child who has a minor sports injury and a plain xray shows lesion
    • Round cell tumors myeloma, lymphoma, metastatic dz, small cell osteosarcoma, Ewing’s sarcoma, infection• <5 yo then think Neuroblastoma• 10-40 yo think of the destructive group (OSA, Ewing’s, infection)• >40 yo think Metastatic Dz, Myeloma, Lymphoma – Note that infection can be seen in any age group
    • Ewing’s Sarcoma• Malignant round cell sarcoma affecting young patients 2-25.• Commonly Diaphyseal• <5 yo, consider leukemia or metastatic neuroblastoma.• >30 yo, consider metastatic carcinoma or lymphoma• Third most common primary sarcoma of bone 1. osteosarcoma 2. Chondrosarcoma 3. Ewings
    • Ewing’s Sarcoma• A type of primitive peripheral neuroectodermal tumor (PNET)• Most common in the 1st and 2nd decades• Preferentially affects whites more than blacks and Asians• 3:2 male to female ratio• Found in the lower extremity more than the upper extremity, but any long tubular bone may be affected – most common sites are the metaphysis and diaphysis of the femur followed by the tibia and humerus• Clinical presentation includes pain and swelling of weeks or months duration Osteomyelitis is often the initial – Erythema and warmth of diagnosis based on fevers, the local area are sometimes seen leukocytosis, anemia and an increased ESR
    • Ewing’s Sarcoma • Xrays: often a central lytic lesion associated with a lamellated or "onion skin" periosteal reaction (caused by and splitting and thickening of the cortex by tumor cells) – "onion-skin" appearance is often followed with a "moth- eaten" or mottled appearance and extension into soft tissue
    • Ewing’s SarcomaImaging:• CT is helpful in defining bone destruction• MRI is essential to elucidate the soft tissue involvement – T1-weighted images low intensity compared to the normal high intensity of bone marrow. On T2 -weighted images the tumor is hyper intense compared to muscle• Increased uptake on bone scan
    • Ewing’s Sarcoma • Histology: densely packed uniform small round blue cells in sheets (+CD99) – Glycogen is present within the cells causing (+) reaction to periodic acid- schiff (PAS) stain – Most are positive with HBA-71 or 0- 13 stain which is an antibody to the product of myc – distinguished from metastatic neuroblastoma by reticulin stain and urine vanillyl mandelic acid and homovanillic acid – Rhabdomyosarcoma is ruled out if the specimen stains negatively with desmin, myoglobin and actin stains – neural origin is supported by electron microscope findings of pseudorosettes – This is further supported by the common finding in Ewings sarcoma and primitive neuroectodermal tumors of choline acetyltransferase and the translocation t(11:22)
    • Ewing’s Sarcoma• Treatment – includes surgery, radiation and multi-drug chemotherapy – XRT or chemo used preoperatively – Adjuvant chemotherapy follows surgery and decreases recurrences• Poor prognostic signs: – increased age – increased ESR at presentation – leukocytosis at presentation• Distinguish from metastatic neuroblastoma by keratin stain
    • Neuroblastoma • The second most common solid malignancy of childhood (after brain tumors) – responsible for ~ 10% of all pediatric neoplasms (and up to 1/2 of malignancies diagnosed in infancy) • predominantly a tumor of early childhood (2/3 dx’d when < 5 yo) • often begins in the nerve tissue of the adrenal glands • Xrays: permeative lesion with medullary bone replacement and variable lysis • Histology: densely cellular sheets of small cells with dark nuclei and scant cytoplasm that have an overall primitive appearance – Rosettes (Homer Wright pseudorosettes) are often found - in them, tumor cells are arranged in circles around a central mass of neuropil • The majority (about 90%) produce catecholamines which are an important diagnostic feature – elevated blood levels of catecholamines and elevated urine levels of metabolites such as vanillymandelic acid VMA and homovanillic acid HVA are present • Metastatic dz = dismal <5% 3 year survival and need to be treated with surgery, chemotherapy, and radiation
    • Osteomyelitis• More common in young• The most common organism in all pts = Staph Remember Neonates- group B strep/E coli, Sickle- Salmonella, shoe Puncture- Pseudomonas, after Surg-Staph epi• Hematogenous osteo occurs most commonly in the distal femur, proximal tibia, proximal femur and proximal humerus• May be caused by direct innoculation secondary to trauma or surgery – 5% of Open frx and 5% of acute OM become chronic OM – Biopsy chronic cases—1% chronic sinuses get SCC (Marjolin’s ulcer)
    • Osteomyelitis• Xrays: Radiolucent and radiodense lesions usually in metaphysis (may cross growth plate into epiphysis) – Chronicmottled bone, periosteal rxn, bony deformation• ―Brodie’s Abscess‖ = Subacute OM—well defined lytic defect and mottled sclerosis of adjacent metaphysis• Sequestrum (dead cortical bone) and Involucrum (new cortical bone b/c periosteal bld supply external)
    • Osteomyelitis• On MRI scan, T1 weighted images demonstrate infection as a low signal with ill defined margins. T2 images show infection as a bright signal..• Histology: 3 featuresmixed cell population, new capillaries and tissue separation (edema)• Rx: Surgical sampling or needle biopsy is necessary for diagnosis – Infected hardware should be removed if the bone is healed and stable – Acute osteomyelitis is treated with irrigation and debridement as necessary, followed by four to six weeks of antibiotics – Chronic osteomyelitis is best treated with thorough debridement, antibiotics, and local flap coverage if necessary
    • Hematopoietic Bone Lesions• Lymphoma• Multiple myeloma• Solitary plasmacytoma• Osteosclerotic myeloma
    • Lymphoma• Bony involvement of Hodgkin’s disease (HD) is between 9.2%-30.3% of patients• Primary Bone Involvement (primary lymphoma of bone) <1% -- Osseous lesions are most commonly due to hematogenous dissemination in stage IV disease or due to direct invasion from an adjacent lymph node leading to focal sclerosis
    • Lymphoma • Xrays: extensive lytic/blastic changes with 25-50% pts having CORITCAL THICKENING (which is also seen in Chondrosarcoma, Pagets, and Chronic OM) • presents in the 3rd or 4th decades or later • The sites of predilection are long bones and primarily diaphyseal affecting the medulla of the bone • Multifocal in older age group • Bone scan is useful and MRI is very sensitive in the localization of subtle marrow disease – hypointense signal on T1 weighted spin echo sequences and high signal on T2 weighted gradient echo sequences
    • Lymphoma• Histology: Bone marrow taken over by lymphocytes of all shapes and sizes (usually large non-cleaved B-cell)• Often, a diagnosis of ―histologic exclusion‖ – Given lytic lesion in pt >40yo on histologic slide see NO glands (mets), NO plasma cells (MM), NO cartilage (CS), and NO storiform pattern (MFH)• Rx: Pts w/ one or multiple bone lesions usually respond well to combined modality treatment, including chemo and local XRT• Surgery should be considered for lesions that present a risk of pathological fracture.
    • Lymphoma• Primary is usually Non-Hodgkins lymphoma• Mottled, blastic bone lesion• VERY hot on bone scan• Histology- mixed round cell inflitrate• Treatment- radiation and chemotherapy• Mets, MM, and Lymphoma usually do not require surgery (except for structural insufficiency)
    • Multiple Myeloma• Plasma cell malignancy in patients >50yo.• Plasma cells produce immunoglobulins• Bone destruction by RANKL• Bisphosphonates reduce the number of skeletal events in patients with MM.• Numerous Punched out lesions• 30% of bone scans are cold: need to get skeletal survey• Serum Protein Electrophoresis (SPEP)- M spike- Immunoglobulin G (50%). Immunoglobulin A (25%)• Treatment is chemo and radiation
    • Multiple Myeloma• Most common primary malignant tumor• Median age: 68 y for men/70 y for women• Involves spine, skull, ribs, sternum and pelvis but may affect any bone with hematopoietic red marrow• Normocytic, normochromic anemia secondary to marrow failure and an increased ESR• SPEP—may see ―goal post sign‖ – Specifically, SPEP>75% sens in dx while UPEP adds ~20%• Single greatest prognostic factor is Renal Function• Median survival 2 yrs
    • Multiple Myeloma• Xrays: diffuse osteopenia with multiple ―punched out‖ radiolucent lesions w/no periosteal reaction• Bone scan can fail to have increased uptake in 25% pts suggesting a skeletal survey should always be done• Histology: Uniform population of monoclonal plasma cells (>30% BM)• Rx: palliative chemo or BMT – Only patients with complete remission experience any bony healing – Bisphosphonates used to inhibit bone resorption – Surgery & XRT 2-3 wks later for impending/path frx
    • Plasmacytoma• A local disease of MM• Treatment is radiation• Better prognosis than MM.
    • Osteosclerotic Myeloma• Poems syndrome• Associated with neuropathy
    • Tumor Like Conditions of Bone• Aneurysmal Bone Cyst• Unicameral Bone Cyst (Simple Bone Cyst)• Histiocytosis (Langerhans’ Cell Histiocytosis• Fibrous Dysplasia
    • Aneurysmal Bone Cyst• Vertebrae and long bones are typical location• Lytic and expansile lesion in patients less than 20 years old.• Fluid-fluid lines on x-ray, better seen on MRI• Will see RBC in histology• Treatment: Curretage and bone graft• 25% recur
    • Aneurysmal Bone Cyst• found most commonly during the second decade• most common location is the metaphysis of the lower extremity long bones (>than the upper extremity) – vertebral bodies also may be involved• 30% have identifiable pre- existing lesion• translocation involving the 16q22 and 17p13 chromosomes has been identified in the solid variant and extraosseous forms of aneurysmal bone cyst
    • Aneurysmal Bone Cyst • Present with PAIN (vs UBC—fracture) • Xrays: purely lytic, expansile, eccentric metaphyseal lesion that erodes cortex (―soap bubble‖) – Fluid-fluid levels • Histology: blood-filled spaces with NO endothelial lining • Rx: biopsy to rule out telengiectatic OSA then extended curettage/grafting – Recurrence statistically related to young age and open growth plates, and may be less likely following wide excision
    • Aneurysmal Bone Cyst • Present with PAIN (vs UBC—fracture) • Xrays: purely lytic, expansile, eccentric metaphyseal lesion that erodes cortex • Fluid-fluid levels on CT/MR • Histology: blood-filled spaces with NO endothelial lining • Rx: biopsy to rule out telengiectatic OSA then extended curettage/grafting – Recurrence statistically related to young age and open growth plates, and may be less likely following wide excision – White space and blood
    • Aneurysmal Bone Cyst• Common in second decade• Most common location is the metaphysis of the lower extremity long bones (>than the upper extremity) – vertebral bodies also may be involved• 30% have identifiable pre- existing lesion• Translocation involving the 16q22 and 17p13 chromosomes has been identified in the solid variant and extraosseous forms of aneurysmal bone cyst• Telangiectatic Osteosarcoma DDX 1/1000
    • Unicameral Bone Cyst • Also known as Simple Bone Cyst • Most commonly found in children between 5- 15 yo (85% pts < 20 yo) • Most common site: prox humerus (50%) followed by prox femur (25%) • Xrays: radiolucent, centered lesion with thinned cortices and metaphyseal expansion to width of physis – ―fallen leaf‖ sign = fx
    • Unicameral Bone Cyst• Usually asymptomatic and only present when pathologic frx occurs• Histology: thin fibrous membrane lines lumen• Rx: aspiration confirmation then steroid injection(s) – If pathologic frxlet heal first before injecting – In lower ext, may consider open curettage/grafting; however, recurrence rates reported up to 40%• MRI all cysts to diagnose fluid inside
    • How to tell UBC from ABC• UBC • ABC – Minimal pain unless frx – Pain even without frx – No h/o trauma unless frx – May have had direct blow – Fluid-filled cavity – Blood-filed cavity – Slightly expansile – Very expansile – Xray with few loculations – Xray with many loculations – MRI with minimal fluid-fluid – MRI with prominent fluid-fluid levels levels – (+)membrane lining – No membrane lining – White space (mesenchymal tissue lining) on path – White space & blood on path
    • Histiocytosis (Langerhans’ cell Histiocytosis• Continum- Eosinophilic granuloma—Hand- Schuller-Christian Disease—Letterer-Siwe disease.
    • Eosinophilic Granuloma• Affects multiple bones and occuring in the youth.• Punched out lesion on x-ray• Vertebra plana• Treament low dose radiation, bone graft with curretage, steroid injection, or observation
    • Eosinophilic Granuloma • part of a spectrum of Langerhans cell histiocytosis, formerly known as histiocytosis X • localized lesion in bone or lung and occurs most commonly in children aged 5 to 10 • makes up 60-80% of all cases of Langerhans cell histiocytosis • uncommon in blacks • found in the skull, mandible, spine and long bones • In spine, may cause vertebra plana (Calve’s disease) • male to female ratio is two to one • Letterer-Siwe disease is a fulminant systemic disease that comprises 10% of Langerhans cell histiocytosis, occurs in children under 3 years old and is rapidly fatal • Hand-Schuller-Christian disease (HSC) is a chronic disseminated form of Langerhan s histiocytosis – occurs in older patients – triad of HSC is diabetes insipidus, exopthalmos and skull lesions
    • Eosinophilic Granuloma• XRAY: well-circumscribed, central radiolucent lesion in metaphysis or diaphysis of long bone with radiodense rim – ―hole within a hole‖ pattern• Histology: sheets of Langerhans cells, multinucleated giant cells, and eosinophils – LC as Eosinophilic histiocytes with coffee bean shape nuclei – identifiable under EM: racket shaped cytoplasmic inclusion bodies called Birbecks granules – Confirmed by CD1a stain• Rx: With localized disease, often a biopsy alone is enough to incite healing – Other treatment modalities of EG include curettage, excision, steroid injection, radiation and observation – Chemotherapy is recommended for systemic disease
    • McCune-Albright syndrome• Precocious puberty• Café au lait spots• Poly-ostotic fibrous dysplasia• Chinese letters histology• Treatment- observe vs. internal fixation• ―Fibrous dysplasia heals with fibrous dysplasia‖
    • Malignant Fibrous Histiocytoma• Most frequent soft tissue tumor in adults• Highest incidence in 5th decade• Found in the extremities 75% of the time (50% lower ext) – In order: Distal femur, prox tibia, prox femur, prox humerus• #1 sarcoma to complicate a pre- existing condition (Paget’s, bone infarct, radiation, AVN, surgery, NOF) – 20% Secondary to another condition – These lesions are ususally more aggressive (presenting as Stage IIb sarcoma)
    • Malignant Fibrous Histiocytoma• Clinically, p/w rapidly enlarging mass• XRAY: extensive, permeative lesion, usually central in metaphyseal or diaphyseal region – ―mottled bone‖ – Associated soft tissue mass• CT scan is helpful in determining cortical erosion• MRI is a must: intermediate T1/high T2• Increased uptake on Bone scan
    • Malignant Fibrous Histiocytoma • Histology: Malignant multi-nucleated giant cells with ―storiform‖ spindle cell fibrogenic areas (create ―cartwheels‖) – Stains (+) for CD68 and Lysosome – 5 subtypes: pleomorphic- storiform (most common 50-60%), myxoid, giant cell, inflammatory, angiomatoid • Rx: wide resection and chemo
    • Bone tumors of unknown origin• Giant cell tumor of bone• Ewing’s Sarcoma (COVERED IN ―ROUND CELL TUMORS‖)• Adamantinoma
    • Giant Cell tumor of Bone• Aggressive benign lesion in 20-40 yos, especially distal radius and proximal tibia.• If ulnar side of distal radius, this osteosarcoma• Imaging- well marginated lytic lesion, subchondral, eccentric, closed physis.• Histology: multi-nucleated giant cells• Treatment- aggressive curretage with bone graft, with a reccurence rate of 2-5%
    • Giant Cell Tumor • XRAY: eccentric, expanding zone of well-defined radiolucency at ends of bones (no reactive sclerosis) • Histology: giant cells with nuclei appearing similar to those of the surrounding nuclei • Rx: Intralesional excision by "extended" curettage is the treatment of choice – Curettage alone is associated with a high recurrence rate, and this can be decreased with the addition of chemical cautery using phenol, multiple freeze-thaw cycles using liquid nitrogen, and treating the walls of the cavity with a high-speed rotary burr – Curettage alone is thought to lead to recurrence in 50% of cases – Recurrence after extended curettage is ~10% – The tumor cavity may be filled with cement or bone graft, according to the surgeons preference
    • Giant Cell Tumor of Bone• occur most often during the third decade• usually found in the long bones, most often the distal femur, proximal tibia, and distal radius• Whether tumor arises in the epiphysis or distal metaphysis is a matter of controversy, but giant cell tumors only occur after the epiphyseal plates have closed – A diagnosis of GCT in a patient with open growth plates should be questioned• Pts p/w progressive pain• 2% met to lungs (10-30% of met pts die) – Therefore, Chest CT for GCT
    • Adamantinoma• Tibia lesion in patients >20 years old, can involve tibia in up to 50%.• Precursor of Osteofibrous dysplasia (Campanacci disease)• Histology- fibrous tissue with epithelioid tissue• Treatment- wide en bloc excision, consider intercalary tibia resection.• 40% recur• Lung mets 25%
    • Adamantinoma• extremely rare, low-grade malignant tumor of epithelial origin• 90% in the diaphysis of the tibia• usually occurs in the 2nd to 5th decade of life• 20% of cases have metastases late in the course of the disease• May mimic osteofibrous dysplasia on xray
    • Adamantinoma • XRAY: multiple eccentric, well- circumscribed lucent defects with intervening sclerosis in the tibia (―soap bubble‖) • Histology: biphasic--blue epithelial nests of cells within fibrous background – Epithelial origin, +keratin • Rx: wide surgical excision – insensitive to radiation and may metastasize to lungs, lymph nodes and abdominal organs by both hematogenous and lymphatic routes – Chemotherapy is not used
    • Chordoma • rare malignant tumor that arises from notochord remnants • account for 1-4% of all bone tumors • occur in older adults with the highest prevalence in the 5th to 7th decade • the ratio of male to female is 2:1 • due to their origin in the notochord, chordomas occur in the mid-line of the axial skeleton – 50% occur in the sacrococcygeal region and 33% occur at the base of the skull – other rare sites include transverse processes of vertebrae and the paranasal sinuses • p/w low back pain, bowel/bladder dysfunction, headaches
    • Chordoma • XRAY: solitary mid-line lesion with bony destruction – often have an accompanying soft tissue mass and ~50% have focal calcifications • CT and MRI scans help demonstrate the soft tissue component, calcifications and epidural extension • Chordomas have reduced uptake on bone scan • Histology: lobules of ―physaliferous‖ cells in fibrous septa – Stain (+) for cytokeratin, S-100, vimentin, and epithelial membrane antigen • Rx: Wide surgical excision is desirable but rarely feasible based on the anatomic location of the tumor – sexual function and sphincter control may be compromised after surgery – Radiation is used if complete resection is impossible – Chemotherapy can be used for late stage disease • Death from Local Complications
    • Testable facts s/p SacrectomyBowel and Bladder Function After Sacral Resection (Todd et al., CORR 4/2002)• Resection Spared Level Normal Bowel Normal Bladder• Bilateral S2-S5 Both S1 0/10 (0%) 0/10 (0%)• Bilateral S3-S5 Both S2 2/5 (40%) 3/12 (25%)• Bilateral S4-S5 Both S3 4/4 (100%) 9/13 (69%)• Variable Unilateral S3 2/3 (67%) 3/5 (60%)• Unilateral S1-S5 Contralateral S1-S5 7/8 (87%) 8/9 (89%)
    • ―Other‖ tumors/lesions OITE Questions
    • OITE QUESTION51. A 12 yo boy sustained a fracture in the distal ulna twice in thepast year. He denies any major fall or injury. A radiograph and abiopsy speciman are shown in Figures 17a and 17b. What is themost likely diagnosis?1. Chondroblastoma2. Giant Cell Tumor3. Unicameral bone cyst4. Fibrous dysplasia5. Nonossifying fibroma
    • Empty White Space
    • OITE QUESTION51. A 12 yo boy sustained a fracture in the distal ulna twice in thepast year. He denies any major fall or injury. A radiograph and abiopsy speciman are shown in Figures 17a and 17b. What is themost likely diagnosis?1. Chondroblastoma2. Giant Cell Tumor3. Unicameral bone cyst4. Fibrous dysplasia5. Nonossifying fibroma
    • OITE QUESTIONA 35-year-old woman has increasing shoulder pain. A radiograph, sagittalT2-weighted MRI scan, and CT scan are shown in Figures 236a through236c. A biopsy specimen is shown in Figure 236d. What is the most likelydiagnosis?1- Infection2- Tuberculosis3- Giant cell tumor4- Aneurysmal bone cyst5- Telangiectatic osteosarcoma
    • Blood and White Space
    • OITE QUESTIONA 35-year-old woman has increasing shoulder pain. A radiograph, sagittalT2-weighted MRI scan, and CT scan are shown in Figures 236a through236c. A biopsy specimen is shown in Figure 236d. What is the most likelydiagnosis?1- Infection2- Tuberculosis3- Giant cell tumor4- Aneurysmal bone cyst5- Telangiectatic osteosarcomaPreferred Response: 4Recommended Reading(s):Kaila R, Ropars M, Briggs TW, et al: Aneurysmal bone cyst of the pediatric shoulder girdle:A case series and literature review. J Pediatr Orthop B 2007;16:429-436.Schwartz HS (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont,IL, American Academy of Orthopaedic Surgeons, 2007, pp 87-102
    • OITE QUESTIONAn 8-year-old girl has had right elbow pain for the past 2 months.Radiographs, MRI scans, and a biopsy specimen are seen inFigures 250a through 250e. What is the most likely diagnosis?1- Osteomyelitis2- Chondroblastoma3- Simple bone cyst4- Eosinophilic granuloma5- Aneurysmal bone cyst
    • Blood and White Space
    • OITE QUESTIONAn 8-year-old girl has had right elbow pain for the past 2 months.Radiographs, MRI scans, and a biopsy specimen are seen inFigures 250a through 250e. What is the most likely diagnosis?1- Osteomyelitis2- Chondroblastoma3- Simple bone cyst4- Eosinophilic granuloma5- Aneurysmal bone cyst
    • OITE QUESTION242. A 55-year-old man has an isolated femoral fracture following a motor vehicle accident. A radiograph is shown in Figure 91. He is hemodynamically stable and his pain is adequately controlled. What is the next most apporpriate step in management?1 – antegrade intramedullary nailing of femur2 – retrograde intramedullary nailing of the femur3 – placement of a distal femoral traction pin4 – wide resection5 – biospy and staging
    • OITE QUESTION242. A 55-year-old man has an isolated femoral fracture following a motor vehicle accident. A radiograph is shown in Figure 91. He is hemodynamically stable and his pain is adequately controlled. What is the next most apporpriate step in management?1 – antegrade intramedullary nailing of femur2 – retrograde intramedullary nailing of the femur3 – placement of a distal femoral traction pin4 – wide resection5 – biospy and staging
    • General Concepts review
    • Staging review• Grade: Histology with aid of radiographic findings and clinical correlation G 1: Low grade, uniform cell type without atypia, few mitoses G 2: High grade, atypical nuclei, mitoses pronounced• Site: T 1: Intracompartmental (Confined within limits of periosteum) T 2: Extracompartmental (Breach in an adjacent joint cartilage, bone cortex (or periosteum) fascia lata, quadriceps, and joint capsule)• Metastasis: M 0: No identifiable skip lesions or distant metastases. M 1: Any skip lesions, regional lymph nodes, or distant mets.• Ennekings Staging System of Malignant Bone Tumors, CORR 1980 Ia Low grade, intracompartmental G1 T1 M0 Ib Low grade, extracompartmental G1 T2 M0 IIa High grade, intracompartmental G2 T1 M0 *IIb High grade, extracompartmental G2 T2 M0 IIIa Low or High grade, intracompart G 1-2 T1 M1 w/ metastases. IIIb Low or High grade, extracompart. G 1-2 T2 M1 w/ metastases. Osteosarcoma Most Commonly Diagnosed at IIB
    • Biopsy Pearls• Do not do the biopsy unless you are the one who is going to take care of the patient – at least talk to the ortho oncologist at your regional referral center before you biopsy• Never use a transverse incision• Hemostasis is vitally important• 2-12 times greater complication if not done at treating hospital• FNA-70-75% accurate• Core needle biopsy 85% accurate• Open Biopsy- 96-98% accurate
    • Tumor Resection Definitions• Intralesional: through the tumor• Marginal: through the reactive zone• Wide: En Blco removal ( Standard)• Radical : Entire compartment removed
    • Pelvic Resections• Internal hemipelvectomy – keep the limb, +/- reconstruct with allograft hemipelvis (often need hemiarthroplasty in addition, as native femoral head may not fit allograft acetabulum, or native FH blood supply may be taken)• External hemipelvectomy – take the limb• Type I – ilium, no reconstruction needed• Type II - periacetabular• Type III – parasymphyseal region• Type IV – involving SI joints/sacrum
    • Immunostains: Bone tumors• S-100 = chordoma, EG• Keratin= Mets, chordoma, adamantinoma• CD1A = EG• CD20 = Lymphoma• CD99 = Ewing’s• Cd138 = Myeloma
    • Ewing’s Sarcoma• Chromosomal translocation T(11:22), found in 90% of patients.• Positive CD 99 reactivity• Positive Vimentin stain (sarcoma stain)• Histology: Monotonous sheets of small smudgy blue cells, pseudorosettes.• Lung mets are common• Treatment- Chemo, resection vs radiation, chemo again.
    • Immunostains: Soft Tissue tumors• Keratin = synovial cell, epitheliod sarcoma• EMA = synovial cell• Smooth muscle actin = leiomyosarcoma• Desmin = Rhabdomyosarcoma• Myoglobin = Rhabdomyosarcoma• S100 = clear cell, nerve sheath tumor• CD34 = Angiosarcoma• Elastin = Elastofibroma
    • Common Chromosomal translocations• T(11:22) = Ewing’s sarcoma• T (X:18) = synovial sarcoma• T (2:13) = alveolar rhabdomyosarcoma• T (12:22) = clear cell sarcoma• T(9:22) = chondrosarcoma• T(12;16) = myxoid liposarcoma
    • Tumor Suppression Genes• RB-1 – Regulates gene expression – Both must be gone (recessive) – 35% of osteosarcomas• P53 - Prevent entrance into S-phase - One bad copy and you have the tumor - 20-65% of Osteosarcomas
    • Oncogenes• Induce uncontrolled growth• Resessive• FAK, erb-2• Chemotherapy for these tumors
    • Chemotherapy• Induces programmed cell death• Eliminated micromets in lungs• Improves survival rate with >95% kill• Reduces recurrence• Bleomycin= concern for pulmonary fibrosis• Adriamycin=concern for cardiomyopathy
    • Immunohistochemistry Review• Keratins: seen in epithelial cells. Classic marker of metastatic carcinomas. Also seen in sarcomas which show some epithelial differentiation including adamantinoma, epithelioid sarcoma (most common sarcoma found in hand), and synovial sarcoma (most common sarcoma found in foot).
    • Immunohistochemistry Review• Vimentin: typical stain of all tumors of mesothelial origin. (+) for almost all sarcomas. (-) in carcinomas.• Desmin: typically found in muscle cells and tumors with myo-differentiaition. These include rhabdomyosarcoma, leiomyosarcoma, and leiomyomas. Occasionally, (+) for desmoids and primitive neuroectodermal tumors.• Actin: as with desmin, indicative of myogenous differentiation.• S-100: most commonly associated with neural, chondroid, or melanocytic differentiation.• Factor VIII related antigen: (+) in lesions with vascular differentiation like hemangiomas.
    • Immunohistochemistry Review• Metastatic cancer Keratin• Lymphoma CD20 (B cell)• Ewing’s CD99• Synovial Sarcoma vimentin, EMA• Nerve tumors S-100• Langerhans cell histiocytosis (EG) S100,CD1A• Chordoma Keratin, S100, vimentin, EMA• Myeloma CD138• Adamantinoma Keratin
    • WARNING: TEST TOPICS• CT Chest/Abd/Pelvis to Stage• Osteosarcoma – Chemo/Surgery/Chemo• Chondrosarcoma - Surgery• Metastatic disease (renal cell bleeds, lung in hand) – Mechanical• Plasmcytoma / Multiple Myeloma - Mechanical• Fibrous Displasia - Mechanical• Enchondroma - Mechanical• Giant Cell Tumor of Bone – Locally aggressive, Mechanical, Mets
    • OITE Questions
    • OITE 2008 – Basic Science / Onc• 15 A 19-year-old man has the mass shown in Figure 5a. MRI reveals a heterogeneous mass. A biopsy specimen is shown in Figure 5b. What is the most likely diagnosis?• 1- Synovial sarcoma• 2- Epithelioid sarcoma• 3- Liposarcoma• 4- Lipoma• 5- Desmoid tumor
    • OITE 2008 – Basic Science / OncQ15. Figs 5a and 5b
    • OITE 2008 – Basic Science / Onc• 15 A 19-year-old man has the mass shown in Figure 5a. MRI reveals a heterogeneous mass. A biopsy specimen is shown in Figure 5b. What is the most likely diagnosis?• 1- Synovial sarcoma• 2- Epithelioid sarcoma• 3- Liposarcoma• 4- Lipoma• 5- Desmoid tumor
    • OITE 2008 – Basic Science / Onc Recognizing synovial sarcoma Grossly -- well circumscribed, firm, gray-pink tumor Radiographically -- focal calcifications Microscopically – Many are histologically similar to synovial tissue and are characterized by a biphasic growth pattern consisting of plump epithelioid cells that form cords, nests, and glandular spaces, and areas of uniform spindle cells (in plump fascicles with hyalinization and distinct lobulation accompanied by mast cells, occasional osseous or cartilaginous metaplasia, and focal whorling) that resemble fibrosarcomas. May be monophasic without epithelial cells. Synovial sarcoma • Most prevalent between 15-40 years of age. • More common in males and tends to affect the extremities (85%-95%) • The natural history of primary synovial sarcoma of the extremity is related to tumor size (> 5 cm) and invasion of bone and neurovascular structures • Treatment: surgical excision with a wide or radical margin and limb preservation when possible; Perioperative radiation if unable to get wide marginLewis JJ, Antonescu CR, Leung DH, et al: Synovial sarcoma: A multivariate analysis of prognostic factors in 112patients with primary localized tumors of the extremity. J Clin Oncol 2000;18:2087-2094.Thompson RC Jr, Garg A, Goswitz J, et al: Synovial sarcoma: Large size predicts poor outcome. Clin Orthop Relat Res2000;373:18-24.
    • OITE 2008 – Basic Science / Onc• 31 A 34-year-old woman has a painful right knee mass. A radiograph, MRI scans, and H & E biopsy specimens (20X and 100X) are shown in Figures 10a through 10d. What is the next most appropriate step in management?• 1- Chemotherapy alone• 2- Arthrotomy with intralesional resection• 3- Extra-articular wide resection• 4- Above-knee amputation• 5- CT of the chest and a bone scan
    • OITE 2008 – Basic Science / OncQ31. Figs 10a & 10b
    • OITE 2008 – Basic Science / Onc• Q31. Figs 10c & 10d
    • OITE 2008 – Basic Science / Onc• A 34-year-old woman has a painful right knee mass. A radiograph, MRI scans, and H & E biopsy specimens (20X and 100X) are shown in Figures 10a through 10d. What is the next most appropriate step in management?• 1- Chemotherapy alone• 2- Arthrotomy with intralesional resection• 3- Extra-articular wide resection• 4- Above-knee amputation• 5- CT of the chest and a bone scan
    • OITE 2008 – Basic Science / Onc• PVNS – PVNS is a proliferation of synovial villi and nodules that may occur locally within a joint or diffusely. Proliferation of synovial tissue can affect the whole of the synovium (diffuse), or a localised area (nodular). – Microscopically – subsynovial nodular proliferation of large round, polyhedral, or spindle cells with prominent cytoplasm and pale nuclei; phagocytic histiocyte-like cells, lipid-laden foam cells and multinucleated giant cells are interspersed with hemosiderin-laden cells (look for iron deposition – pathopneumonic of PVNS) – Most common presentation – Progressive and insidious onset of pain and swelling of a joint • Knee is most commonly affected, but it is also seen in the hip and shoulder • Typically in 3rd to 4th decade – Treatment – Complete open or arthroscopic synovectomy
    • OITE 2008 – Basic Science / Onc• A 10-year-old girl has had pain in the right hip for the past 2 weeks. She denies any history of trauma or other diseases. Imaging studies and biopsy specimens are shown in Figures 17a through 17e. What is the most appropriate treatment?• 1- Preoperative chemotherapy, wide resection, and postoperative chemotherapy• 2- Hip arthrotomy, irrigation, and intravenous antibiotics for 6 weeks• 3- In situ pinning with a 7.3-mm cannulated screw• 4- Radiation therapy• 5- Crutch walking and nonsteroidal anti-inflammatory drugs
    • OITE 2008 – Basic Science / OncQ46. Fig 17a
    • OITE 2008 – Basic Science / OncQ46. Fig 17 b & c
    • OITE 2008 – Basic Science / OncQ46. Fig 17 d & e
    • OITE 2008 – Basic Science / Onc• A 10-year-old girl has had pain in the right hip for the past 2 weeks. She denies any history of trauma or other diseases. Imaging studies and biopsy specimens are shown in Figures 17a through 17e. What is the most appropriate treatment?• 1- Preoperative chemotherapy, wide resection, and postoperative chemotherapy• 2- Hip arthrotomy, irrigation, and intravenous antibiotics for 6 weeks• 3- In situ pinning with a 7.3-mm cannulated screw• 4- Radiation therapy• 5- Crutch walking and nonsteroidal anti-inflammatory drugs
    • OITE 2008 – Basic Science / Onc• Recognizing Osteosarcoma – Grossly: large tender soft tissue mass typically over metaphyses of long bones – Radiologically: destruction of the normal trabecular bone pattern, indistinct margins, no endosteal bone response, a mixture of radiodense and radiolucent areas, with periosteal new bone formation, lifting of the cortex, and Codmans triangle – Microscopically: malignant sarcomatous stroma, w/ production of osteoid and bone • 3 main types: osteoblastic, chondroblastic, fibroblastic
    • OITE 2008 – Basic Science / Onc• 53 A 5-year-old girl has knee pain, a low grade fever, and a limp. A radiograph and MRI scan are shown in Figures 19a and 19b, and a biopsy specimen is shown in Figure 19c. Molecular genetic analysis identifies an EWS-FLI1 gene rearrangement. In addition to a CT scan of the chest and a bone scan, what other study is indicated for complete tumor staging?• 1- Spinal tap• 2- MRI of the brain• 3- Bone marrow biopsies• 4- Sentinel lymph node biopsy• 5- Serum alkaline phosphatase
    • OITE 2008 – Basic Science / OncQ53. Figs 19 a – c.
    • OITE 2008 – Basic Science / Onc• 53 A 5-year-old girl has knee pain, a low grade fever, and a limp. A radiograph and MRI scan are shown in Figures 19a and 19b, and a biopsy specimen is shown in Figure 19c. Molecular genetic analysis identifies an EWS-FLI1 gene rearrangement. In addition to a CT scan of the chest and a bone scan, what other study is indicated for complete tumor staging?• 1- Spinal tap• 2- MRI of the brain• 3- Bone marrow biopsies• 4- Sentinel lymph node biopsy• 5- Serum alkaline phosphatase
    • OITE 2008 – Basic Science / Onc• Recognizing Ewing’s Sarcoma Grossly – rapid growing painful mass that tender on exam Radiographically –highly aggressive appearance with permeative or ―moth-eaten‖ bone destruction occurring in the metadiaphysis of long bones. Codman’s triangle often present. Periosteal reaction (classically: onion skin appearance) and soft tissue mass without calcifications are common. Microscopically – Small blue cell tumor; can’t differentiate from other small blue cell tumors therefore need BM Bx• Simon MA, Springfield D (eds): Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott-Raven, 1998, pp 287-297.• Carvajal R, Meyers P: Ewings sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am 2005;19:501-525.
    • 85. A 15 yo cross country runner reports shin pain that is present during running, at rest, and at night. The pain is relieved by ibuprofen. Radiographs, a bone scan, and a CT scan are shown in Figures 32a through 32c. What is the most likely diagnosis?1. Cortical desmoid2. Osteoblastoma3. Stress fracture4. Enchondroma5. Osteoid osteoma
    • 85. A 15 yo cross country runner reports shin pain that is present during running, at rest, and at night. The pain is relieved by ibuprofen. Radiographs, a bone scan, and a CT scan are shown in Figures 32a through 32c. What is the most likely diagnosis?1. Cortical desmoid2. Osteoblastoma3. Stress fracture4. Enchondroma5. Osteoid osteoma
    • 92. A 45 yo man has a long-standing history of an ankle mass. He now reports that the mass has enlarged and become quite painful over the past several months. A radiograph and axial MRI scans are shown in Figures 35a and 35b. What is the next most appropriate step in management?1. Below knee amputaton2. Neoadjuvant chemotherapy3. Radiation Therapy4. Chest imaging and a bone scan5. Limb salvage with an allograft arthrodesis
    • 92. A 45 yo man has a long-standing history of an ankle mass. He now reports that the mass has enlarged and become quite painful over the past several months. A radiograph and axial MRI scans are shown in Figures 35a and 35b. What is the next most appropriate step in management?1. Below knee amputaton2. Neoadjuvant chemotherapy3. Radiation Therapy4. Chest imaging and a bone scan5. Limb salvage with an allograft arthrodesis
    • 143. An 11 yo boy falls during a soccer game and is unable to finish the match. The radiographs shown in figures 62a and 62b reveal a radiolucent lesion of the tibia but no fracture. What is the most appropriate management?1. Repeat radiographs in 3 to 6 months2. Needle biopsy and radiofrequency ablation under CT direction3. MRI, bone scane, chest CT, biopsy, and chemotherapy4. Curettage and bone grafting5. En bloc resection with autogenous tricortical bone grafting
    • 143. An 11 yo boy falls during a soccer game and is unable to finish the match. The radiographs shown in figures 62a and 62b reveal a radiolucent lesion of the tibia but no fracture. What is the most appropriate management?1. Repeat radiographs in 3 to 6 months2. Needle biopsy and radiofrequency ablation under CT direction3. MRI, bone scane, chest CT, biopsy, and chemotherapy4. Curettage and bone grafting5. En bloc resection with autogenous tricortical bone grafting
    • 159. A 12 yo boy is seen in the ED at 11pm with severe thigh pain. A radiograph is shown in Figure 69. A decision is made to transfer his care to the local orthopaedic oncologist in the morning. What should be done prior to transfer?1. Splinting and pharmacologic pain control2. Biopsy3. Spanning external fixator4. Femoral traction pin5. Crutch training
    • 159. A 12 yo boy is seen in the ED at 11pm with sever thigh pain. A radiograph is shown in Figure 69. A decision is made to transfer his care to the local orthopaedic oncologist in the morning. What should be done prior to transfer?1. Splinting and pharmacologic pain control2. Biopsy3. Spanning external fixator4. Femoral traction pin5. Crutch training
    • OITE 2008—Orthopedic Diseases 162. A 34-year-old man has had progressive back, rib, and right thigh pain for the past 2 months. A bone scan is shown in figure 72. Further work-up includes routine laboratory analysis, including PSA; radiographs of the involved bones, and CT of the chest, abdomen, and pelvis. A primary is not discovered. The prognosis for this patient is most similar to patients with bone metastases from which of the following primary sites? 1. Breast 2. Prostate 3. Kidney 4. Thyroid 5. Lung
    • OITE 2008—Orthopedic DiseasesFigure 72:
    • OITE 2008—Orthopedic Diseases 162. A 34-year-old man has had progressive back, rib, and right thigh pain for the past 2 months. A bone scan is shown in figure 72. Further work-up includes routine laboratory analysis, including PSA; radiographs of the involved bones, and CT of the chest, abdomen, and pelvis. A primary is not discovered. The prognosis for this patient is most similar to patients with bone metastases from which of the following primary sites? 1. Breast 2. Prostate 3. Kidney 4. Thyroid 5. Lung
    • OITE 2008—Orthopedic DiseasesSchwartz HS (ed): OKU: MSK Tumors 2. Rosemont, IL, American. AAOS, 2007, pp 353-357.―In 3-4% of patients with metastatic carcinoma, the primary site of origin is unknown.‖Breast, lung, thyroid, kidney, prostate classically met to bones (―BLT w/ Kosher Pickle‖ if you recall USMLE Step I).―If the lesion appears to primarily involve the cortex of a bone, or is located distal to the knee or elbow, it is more likely to originate from an occult primary carcinoma of the lung than from other sites.‖―In one study, radiographs of the chest identified the lung as the primary tumor site in 43% of patients.‖
    • OITE 2008—Orthopedic Diseases167. A 54-year-old man with a history of alcoholism and prostate carcinoma has mild knee pain after falling. Radiographs are shown in Figures 74a nd 74b. What is the most likely radiographic diagnosis? 1. Chondrosarcoma 2. Bone infarct 3. Osteogenic sarcoma 4. Metastatic carcinoma of uncertain origin. 5. Metastatic prostate carcinoma.
    • OITE 2008—Orthopedic DiseasesFigure 74a: Figure 74b:
    • OITE 2008—Orthopedic Diseases167. A 54-year-old man with a history of alcoholism and prostate carcinoma has mild knee pain after falling. Radiographs are shown in Figures 74a nd 74b. What is the most likely radiographic diagnosis? 1. Chondrosarcoma 2. Bone infarct 3. Osteogenic sarcoma 4. Metastatic carcinoma of uncertain origin. 5. Metastatic prostate carcinoma.
    • OITE 2008—Orthopedic DiseasesBone Infarction vs. Enchondroma• Linear or serpiginous pattern more suggestive of infarction; popcorn or floculent pattern w/ arcs/swirls matrix suggestive of chondroid matrix/enchondroma.Bone Infarct Enchondroma
    • OITE 2008—Orthopedic Diseases173. A 24-year-old man has a painful mass on his left humerus. A radiograph, MRI scan, and a biopsy specimen are shown in Figures 77a through 77c. What is the most likely diagnosis? 1. Myositis ossificans 2. Osteochondroma 3. Periosteal osteosarcoma 4. Periosteal chondroma 5. Sarcoma
    • OITE 2008—Orthopedic DiseasesFigure 77a: Figure 77b:
    • OITE 2008—Orthopedic DiseasesFigure 77c:
    • OITE 2008—Orthopedic Diseases173. A 24-year-old man has a painful mass on his left humerus. A radiograph, MRI scan, and a biopsy specimen are shown in Figures 77a through 77c. What is the most likely diagnosis? 1. Myositis ossificans 2. Osteochondroma 3. Periosteal osteosarcoma 4. Periosteal chondroma 5. Sarcoma
    • OITE 2008—Orthopedic DiseasesPeriosteal chondroma: atlasgeneticsoncology.orgPeriosteal chondroma is a painful cartilaginous lesion that arises from surface of cortex deep to the periosteum, producing broad based cartilaginous mass that may extend into soft tissues; often develops after adolescence. Characteristically erodes and induces sclerosis of the contiguous cortical bone.Pathology: It persists as mass of mature cartilage. Low power microscopy shows well circumscribed lobulated hyaline masses. Cellularity can vary, from hypo- to hyper-cellularity. The cartilage looks more active than enchondroma and the lesion may be confused with chondrosarcoma.
    • OITE 2008—Orthopedic Diseases174. A 29-year-old woman has a right shoulder mass. Figures 78a through 78c show the radiograph, CT scan, and H&E biopsy specimen. These findings are associated with which of the following conditions? 1. Multiple hereditary exostoses 2. Hyperparathyroidism 3. Renal failure 4. Retinoblastoma 5. Hemangiomatosis
    • OITE 2008—Orthopedic DiseasesFigure 78a: Figures 78b:
    • OITE 2008—Orthopedic DiseasesFigure 78c:
    • OITE 2008—Orthopedic Diseases174. A 29-year-old woman has a right shoulder mass. Figures 78a through 78c show the radiograph, CT scan, and H&E biopsy specimen. These findings are associated with which of the following conditions? 1. Multiple hereditary exostoses 2. Hyperparathyroidism 3. Renal failure 4. Retinoblastoma 5. Hemangiomatosis
    • OITE 2008—Orthopedic Diseases204. A 60-year-old man has left shoulder pain. A review of systems reveals hematuria. A radiograph of the affected area is shown in Figure 89a, and MRI scans are shown in Figures 89b and 89c. Examination reveals a pulsatile mass. CT of the chest, abdomen, and pelvis is likely to reveal which of the following findings? 1. Spiculated lung mass 2. Thickened colon 3. Renal mass 4. Hilar adenopathy 5. Prostate irregularity
    • OITE 2008—Orthopedic DiseasesFigure 89a: Figure 89b:
    • OITE 2008—Orthopedic DiseasesFigure 89c:
    • OITE 2008—Orthopedic Diseases204. A 60-year-old man has left shoulder pain. A review of systems reveals hematuria. A radiograph of the affected area is shown in Figure 89a, and MRI scans are shown in Figures 89b and 89c. Examination reveals a pulsatile mass. CT of the chest, abdomen, and pelvis is likely to reveal which of the following findings? 1. Spiculated lung mass 2. Thickened colon 3. Renal mass 4. Hilar adenopathy 5. Prostate irregularity
    • OITE 2008—Orthopedic DiseasesRougraff BT: Evaluation of the patient with carcinoma of unknown origin metastatic to bone. Clin Orthop Relat Res 2003;415:S105-S109.Breast, lung, thyroid, kidney, prostate classically met to bones (―BLT w/ Kosher Pickle‖ if you recall USMLE Step I).• This patient has hematuria, which immediately raises the suspicion of renal cell carcinoma.• Renal cell is also very vascular and therefore a large lesion can be pulsatile.• Note that according to above reference, most mets to bone of unknown origin are either lung (most common) or renal, possibly because these organs are harder to examine on physical exam or tumors may grow larger before becoming symptomatic.
    • OITE 2008 Orthopaedic Diseases214. A 43-year-old woman reports a 2-month history of increasing knee pain. She has no history of malignancy. Radiographs, MRI scans, and a biopsy specimen are showin in Figures 92a through 92e. What is the most likely diagnosis? 1. Chordoma 2. Giant cell tumor 3. Metastatic carcinoma 4. Osteosarcoma 5. Aneurysmal bone cyst
    • OITE 2008 Orthopaedic Diseases 214. A 43-year-old woman reports a 2-month history of increasing knee pain. She has no history of malignancy. Radiographs, MRI scans, and a biopsy specimen are showin in Figures 92a through 92e. What is the most likely diagnosis? 1. Chordoma 2. Giant cell tumor (Correct location but no giant cells) 3. Metastatic carcinoma 4. Osteosarcoma 5. Aneurysmal bone cyst1. Dorfman HD, Czerniak B: Bone Tumors. St Louis, MO, Mosby, 1998, pp 1009-1029.2. Marcove RC, Arlen M: Atlas of Bone Pathology. Philadelphia, PA, JB Lippincott, 1992,pp 522-530.
    • OITE 2008 Orthopaedic Diseases 1. Chordoma (Wrong Location, Usually in Sacrum, base of skull and rare spine; Correcta age; malignant slow growing neoplasm of notochord remnant; Physaliphorous Cells – Large cells with central nuclei, numerous vacuoles in eosinophilic cytoplasm) 2. Giant cell tumor (Correct location but no giant cells) 3. Metastatic carcinoma – (Pathology shows clear cell variant of Renal Carcinoma; Mets are the most common diagnosis; just have to know it is not the other answers) 4. Osteosarcoma (Not the correct age; Usually does not cross the physis; No osteoid on path) 5. Aneurysmal bone cyst (Common in spine and flat bones although 15% occur in the femur; Large spaces marked by septa filled with blood; Giant Cells may be present; Occasional osteoclast lining of septa)1. Dorfman HD, Czerniak B: Bone Tumors. St Louis, MO, Mosby, 1998, pp 1009-1029.2. Marcove RC, Arlen M: Atlas of Bone Pathology. Philadelphia, PA, JB Lippincott, 1992,pp 522-530.
    • OITE 2008 Orthopaedic Diseases226. A 56-year-old man with a history of neurofibromatosis reports burning pain in his foot. An MRI scan of the thigh reveals the mass shown in Figure 96A. A biopsy is most likely to reveal the histology shown in which of the following figures? 1. Figure 96b 2. Figure 96c 3. Figure 96d 4. Figure 96e 5. Figure 96f
    • 1. Figure 96b2. Figure 96c3. Figure 96d4. Figure 96e5. Figure 96f
    • OITE 2008 Orthopaedic Diseases 226. A 56-year-old man with a history of neurofibromatosis reports burning pain in his foot. An MRI scan of the thigh reveals the mass shown in Figure 96A. A biopsy is most likely to reveal the histology shown in which of the following figures? 1. Figure 96b 2. Figure 96c 3. Figure 96d 4. Figure 96e 5. Figure 96f1. Weiss SW, Goldblum JR: Benign tumors of peripheral nerves, in Enzinger FM, Weiss SW(eds): Soft Tissue Tumors. St Louis, MO, Mosby, 2001, pp 1111-1207.2. Schwartz HS (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors 2. Rosemont,IL, American Academy of Orthopaedic Surgeons, 2007, pp 273-275
    • 1. Round blue cellswith myxoid featurescan been seen intypes ofchondrosarcoma2. Osteoid withmalginant spindle cellstroma seen inOsteosarcoma3. Wavy Spindle Cellsare seen inNeurofibromas4. No stroma and lowcystoplasm to nuclearration can be seen inEwing’s Scarcoma5. Vacuoles can beseen in Chordomasand Fatty Tumors
    • OITE 2008 Orthopaedic Diseases236. A 28-year-old woman notes a growing mass in her hand for the past several weeks. Examination reveals a nontender mass in her palm with no skin changes or ipsilatearl adenopathy. An axial MRI scan is shown in Figure 98a and a biopsy specimen is shown in Figure 98b. Immunohistochemistry analysis is positive for epithelial membrane antigen, cytokeratin and sporadic S-100. What is the most likely diagnosis? 1. Glomus tumor 2. Squamous cell carcinoma 3. Ganglion 4. Giant cell tumor of the tendon sheath 5. Synovial sarcoma
    • OITE 2008 Orthopaedic Diseases 236. A 28-year-old woman notes a growing mass in her hand for the past several weeks. Examination reveals a nontender mass in her palm with no skin changes or ipsilatearl adenopathy. An axial MRI scan is shown in Figure 98a and a biopsy specimen is shown in Figure 98b. Immunohistochemistry analysis is positive for epithelial membrane antigen, cytokeratin and sporadic S-100. What is the most likely diagnosis? 1. Glomus tumor 2. Squamous cell carcinoma 3. Ganglion 4. Giant cell tumor of the tendon sheath 5. Synovial sarcoma1. Weiss SW, Goldblum JR, et al: Malignant soft-tissue tumors of uncertain type, in Strauss M,Gery L (eds): Enzinger and Weisss Soft Tissue Tumors, ed 4. St Louis,MO, Mosby, 2001, pp 1483-1509.2. Pradhan A, Cheung YC, Grimer RJ, et al: Soft-tissue sarcomas of the hand: Oncologicaloutcome and prognostic factors. J Bone Joint Surg Br 2008;90:209-214.
    • OITE 2008 Orthopaedic Diseases1. Glomus tumor – related to glomus bodies which help Giant Cell Tumor ofwith temperature regulation, paroxysmal pain, worse tendon sheathwith cold, Dx with MRI2. Squamous cell carcinoma – very rare in the hand,path would likely have keratinized layer of skin, will notbe the OITE answer3. Ganglion – lesion not associated with, MRI showshomogenous fluid filled cystic structure www.bweems.com/gctten.html4. Giant cell tumor of the tendon sheath – Benign lesionassociated with tendon sheath, giant cells are usallypresent on path, must differenciat from syncovial cell Synovial Sarcomasarcoma on path, treatment is marginal excision, 30%reoccur5. Synovial sarcoma – S-100+, EMA and Cytokeratin +,Spindle cells on path, Associate with tendon sheaths,80% occur in the extremities, used to be calledtendosynovial sarcoma
    • OITE 2008 Orthopaedic Diseases252. Figure 102 shows the radiograph of a 40-year-old man who reports worsening knee pain and a limp. A bone scan reveals no other sites of disease. What is the most likely diagnosis? 1. Chondroblastoma 2. Low-grad intramedularry osteogenic sarcoma 3. Giant cell tumor 4. Ewing’s sarcoma 5. Enchondroma
    • OITE 2008 Orthopaedic Diseases 252. Figure 102 shows the radiograph of a 40-year-old man who reports worsening knee pain and a limp. A bone scan reveals no other sites of disease. What is the most likely diagnosis? 1. Chondroblastoma (Patient is too old, diagnosed < 20 y/o) 2. Low-grade intramedularry osteogenic sarcoma (Does not cross the physis) 3. Giant cell tumor (Correct age, crosses the physis) 4. Ewing’s sarcoma (Does not cross the physis) 5. Enchondroma (Does not cross the physis)1. Turcotte RE: Giant cell tumor of bone. Orthop Clin North Am 2006;37:35-51.2. Greenspan A, Jundt G, Remagen W (eds): Differential Diagnosis in Orthopaedic Oncology,ed 2. Philadelphia, PA, Lippincott Williams & Wilkins, 2007, pp 387-457.
    • OITE 2008 Orthopaedic Diseases273. A 28-year-old man with a history of osteogenic sarcoma 10 years ago was treated with standard multi-agent chemotherapy that included doxorubicin. Currently, he is disease free and needs to undergo revision of a distal femur replacement. What test should be performed as part of his preop work-up? 1. Liver ultrasound 2. Echocardiography 3. Bone densitometry 4. Bone marrow aspiration 5. Skin tuberculin testing
    • OITE 2008 Orthopaedic Diseases273. A 28-year-old man with a history of osteogenic sarcoma 10 years ago was treated with standard multi-agent chemotherapy that included doxorubicin. Currently, he is disease free and needs to undergo revision of a distal femur replacement. What test shoulder be performed as part of his preop work-up? 1. Liver ultrasound (many are hepatotoxic, follow with LFT’s, liver mets response to chemo can be followed with US) 2. Echocardiography (Doxorubicin is cardiotoxic) 3. Bone densitometry (Methotrexate) 4. Bone marrow aspiration (L-phenylalanine mustard (L-PAM)  leukemia) 5. Skin tuberculin testing – can be offered before chemo so prophylaxis can be started before they are immunocompromized
    • OITE 2008—Science136. Which of the following is diagnosed by urine protein electrophoresis?1- Monoclonal heavy chains of myeloma2- Monoclonal light chains of myeloma3- Diffuse B-cell lymphoma4- Primary lymphoma of bone5- Amyloidosis
    • OITE 2008—Science136. Which of the following is diagnosed by urine protein electrophoresis?1- Monoclonal heavy chains of myeloma2- Monoclonal light chains of myeloma3- Diffuse B-cell lymphoma4- Primary lymphoma of bone5- AmyloidosisRECOMMENDED READINGS:Dorfman HD, Czerniak B: Bone Tumors. St Louis, MO, Mosby, 1998, pp 664-728.
    • Explanation• Myeloma is an overproduction of Plasma cells ( B-cells) overproducing one kind of antibody.• These antibodies are composed of light- chained and heavy-chained parts; the light-chained parts can be detected in high quantities in the urine, called Bence-Jones proteins, and are diagnositic for myeloma.
    • OITE 2009 – Basic Science & Tumors142. What predominant cell type is typically seen on a biopsy specimen in a patient with multiple myeloma? 1. Spindle cells 2. Giant cells 3. Osteoclasts 4. Lymphocytes 5. Plasma cells
    • OITE 2009 – Basic Science & Tumors142. What predominant cell type is typically seen on a biopsy specimen in a patient with multiple myeloma? 1. Spindle cells 2. Giant cells 3. Osteoclasts 4. Lymphocytes 5. Plasma cells
    • OITE 2009 – Basic Science & TumorsReference: Fletcher C, Unni K, Mertens F (eds): Pathology and Genetics of Tumors of Soft Tissue and Bone.Multiple myeloma-characteristic cell = plasma cell-most common neoplasm of bone-6th & 7th decades of life-characterized by osteolytic lesions, bone pain, hypercalcemia, monoclonal gammopathy, amyloid deposits (eg in kidney) Plasma cells in multiple myeloma-targets = vertebrae, ribs, skull, pelvis, femur, clavicle, scapula-diagnose w/ bone marrow aspirate; often cold on bone scan-Poor prognosis (median survival 3 years)
    • OITE 2009 – Basic Science & Tumors156. Cytogenetic analysis is a useful approach for detecting which of the following genetic abnormalities? 1. Point mutations 2. Frame-shift mutations 3. Microsatellite instability 4. Translocations 5. DNA cross-linking
    • OITE 2009 – Basic Science & Tumors156. Cytogenetic analysis is a useful approach for detecting which of the following genetic abnormalities? 1. Point mutations 2. Frame-shift mutations 3. Microsatellite instability 4. Translocations 5. DNA cross-linking
    • 51. A 12 yo boy sustained a fracture in the distal ulna twice in thepast year. He denies any major fall or injury. A radiograph and abiopsy speciman are shown in Figures 17a and 17b. What is themost likely diagnosis?1. Chondroblastoma2. Giant Cell Tumor3. Unicameral bone cyst4. Fibrous dysplasia5. Nonossifying fibroma
    • 51. A 12 yo boy sustained a fracture in the distal ulna twice in thepast year. He denies any major fall or injury. A radiograph and abiopsy speciman are shown in Figures 17a and 17b. What is themost likely diagnosis?1. Chondroblastoma2. Giant Cell Tumor3. Unicameral bone cyst4. Fibrous dysplasia5. Nonossifying fibroma
    • UBCs1. Occur in young pts2. Present with pain/path fx after minor trauma3. XRAY: Central, lytic, cortical thinning and expansion but not usually beyond width of physis, fallen leaf sign4. PATH: Thin fibrous membrane, giant cells, hemosiderin, lipophages, chronic inflammation5. TREATMENT: Steroid injection vs. curettage & packing if in high risk location (prox femur)
    • 104. Synovial sarcoma is closely associatedwith translocation of which of the followinggenes?1 – BCR-ABL2 – EWS-FLI13 – PDGF-COL1A14 – SYT-SSX5 – PAX3-FKHR
    • 104. Synovial sarcoma is closely associatedwith translocation of which of the followinggenes?1 – BCR-ABL = (CML)2 – EWS-FLI1 = (Ewings)3 – PDGF-COL1A1 = (DermatofibrosarcomaProtuberans)4 – SYT-SSX5 – PAX3-FKHR = (Alveolarrhabdomyosarcoma)
    • 251.Which of the following soft tissue sarcomas is most common in children?1 – epitheloid sarcoma2 – rhabdomyosarcoma3 – synovial sarcoma4 – liposarcoma5 – extraskeletal Ewing’s sarcoma
    • 251.Which of the following soft tissue sarcomas is most common in children?1 – epitheloid sarcoma2 – rhabdomyosarcoma3 – synovial sarcoma4 – liposarcoma5 – extraskeletal Ewing’s sarcoma
    • • Five most common malignant bone tumors in adults >50 – Metastases (PTBLK) – Lymphoma – Myeloma (most common primary bone tumor) – Chondrosarcoma (most common sarcoma age>50) – Osteosarcoma (most common sarcoma)• Ewings is most common malignant bone tumor in children/adolescent• Rhabdomyosarcoma is most common soft tissue sarcoma in children
    • 65. For which of the following skeletal tumors is radiation therapy routinely used for definitive local control? 1- Osteogenic sarcoma 2- Ewing’s sarcoma 3- Chondrosarcoma 4- Pleomorphic sarcoma 5- Paget’s sarcoma
    • 65. For which of the following skeletal tumors is radiation therapy routinely used for definitive local control? 1- Osteogenic sarcoma 2- Ewing’s sarcoma 3- Chondrosarcoma 4- Pleomorphic sarcoma 5- Paget’s sarcoma
    • Question 232 -What is the most current recommendation for definitive treatment of a 15 year-old boywho has a high grade osteosarcoma of the distal femur? 1. Surgical resection only 2. Raditation therapy only 3. Radiation therapy and surgical resection 4. Chemotherapy only 5. Chemotherapy and surgical resection
    • Answer: 5Chemotherapy and surgical resection combined has lead to a >80% 5 year survival rate.Surgical restriction alone has lead to a high recurrence and metastatic rates and poor(17.4%) survival rates. Radiation therapy alone did not control local recurrences andpulmonary metastasis. radiation and surgery also had high local recurrences andmetastatic rates. Neoadjunctive chemotherapy has provided many improvements intreatment including elimination of micro metastases, necrosis of the primary tumor,reduction of tumor size and neovasculization and helps prevent local recurrences.Complete surgical resection is the mainstay of treatment of osteogenic sarcoma, theaddition of neoadjunctive chemotherapy has proven to be the most effective.
    • Question 228 -Which of the following terms best describe most osteosarcomas at the time ofdiagnosis? 1. Metastatic 2. Low-grade, intracompartmental 3. Low-grade, extracompartmental 4. High-grade intracompartmental 5. High-grade extracompartmental
    • Answer: 5DISCUSSION: Osteosarcoma is a difficult disease to cure because it is predominantlyhigh-grade and extracompartmental at the time of presentation, 90% stage IIB. Thecommon presentation is child or young adult with complaints of pain around the knee.The proximal femur and humerus, as well as, the pelvis are not uncommon sites. Pre-operative multiagent chemotherapy is given for 8-12 weeks and then staging studies areperformed. Resection, if possible, is then performed and maintenance chemotherapy isgiven for 6-12 months. Long term survival has increased from 10-20% to 60-70%, withincreased limb salvage.REFERENCES: Enneking WF, Spanier SS, Goodman MA. Clin Orthop 1980;153:106-20.This question deals with the surgical staging of musculoskeletal tumors:STAGE GRADE SITEIA LOW INTRACOMPARTMENTALIB LOW EXTRACOMPARTMENTALIIA HIGH INTRACOMPARTMENTALIIB HIGH EXTRACOMPARTMENTALIII ANY METSUpon diagnosis, most osteosarcomas are high grade, extracompartmental lesions, (stage IIB).
    • Question 186 -A 55 year old man with metastatic prostate cancer has a painful lesion of the midshaftof the humerus in which approx. 75% of the cortex is involved. Management shouldconsist of. 1. an incisional biopsy. 2. a humeral cuff and a sling 3. closed interlocking nailing 4. radiation therapy to the humerus 5. plate fixation with bone fixation
    • Answer: 3Redmond reviewed 13 patients who had 16 pathological fractures of the shaft of thehumerus secondary to metastatic disease. All but one fracture was stabilized withclosed IM nailing. 14 extremities returned to nearly normal function in 3 weeks. Theconclusion stated that IM nailing of the humerus for pathological fractures providesimmediate stability and can be accomplished with a closed technique, brief operativetime, minimum morbidity, with a resultant early return of function to the extremity.
    • Question 89 -A 14-year-old boy has a 4-month history of aching pain in the distal thigh.Examination reveals a mass in the distal thigh. Figure 25a shows a plain radiograph,Figures 25b and 25c show MRI images, Figure 25d shows a bone scan, and Figure 25eshows a CT scan of the chest. The most likely diagnosis and Musculoskelatal TumorSociety (Enneking) stage is 1. osteosarcoma, stage IIB 2. osteosarcoma, stage III 3. parosteal osteosarcoma, stage IIB 4. periosteal osteosarcoma, stage IIB 5. periosteal osteosarcoma, stage III
    • Answer: 2Osteosarcoma commonly occurs about the knee in children and young adults. Patients present primarily withpain. More than 90% of the intramedullary osteosarcomas are high grade and penetrate the cortex early toform a soft tissue mass. About 10-20% of patients have pulmonary metastases at presentation. Plainradiographs demonstrate a lesion in which there is both bone destruction and bone formation. MRI and CTscans are useful for defining the anatomy of the lesion in regard to intramedullary extension, involvement ofneurovascular structures, and muscle invasion.Parosteal Osteosarcoma commonly occurs at the posterior aspect of the distal femur, proximal tibia, andproximal humerus. Patients present with a painless mass. The radiographic appearance is characteristic inthat it demonstrates a heavily ossified, often lobulated mass arising form the cortex.Periosteal Osteosarcoma is a rare form of osteosarcoma and occurs most commonly in the diaphysis of longbones (femur and tibia). The radiographic appearance is fairly constant; a sunburst type lesion rests on asaucerized cortical depression.Staging system of the Musculoskelatal Tumor Society (Enneking System)Stage DescriptionI-A Low grade, intracompartmental, no metastasesI-B Low grade, extracompartmental, no metastasesII-A High grade, intracompartmental, no metastasesII-B High grade, extracompartmental, no metastasesIII-A Any grade, intracompartmental, with metastasesIII-B Any grade, extracompartmental, with metastasesFigure 25a demonstrates an intramedullary lesion, Figures 25b-c demonstrate a large lesion extending intothe soft tissue, and figure 25e demonstrates lung metastasis. With mets it must be a grade III and theradiographic appearance is typical for osteosarcoma.
    • Question 71A 12 year old girl has a Ewing’s sarcoma of the proximal fibula with no metastaticdisease or neurovascular involvment. Treatment should include 1. radiation therapy. 2. chemotherapy. 3. surgical resection. 4. radiation therapy and surgical resection. 5. chemotherapy and surgical resection.
    • Answer: 5 Ewings sarcoma is the second most common primary bone tumor in children.It usually occurs in the first or second decades of life. Radiographically it has a highlyaggressive appearance with permeative bone destruction occurring in the diaphysis oflong bones. It may also occur, however, in flat bones. Periosteal reaction and soft tissuemass without calcifications are common. The periosteal reaction may have a sunburst oronion skin appearance. Radiographic differential diagnosis includes histiocytosis X,osteomyelitis, osteosarcoma, and medullary neuroblastoma. Historically, the 5 year survival rate of the this tumor was found to be about 5-10%. In the past, the use of 5000-6000cGY was utilized to treat the entire bone thatwas affected. This provided good local control of the disease, but it did not preventpulmonary metastasis. Also, the malice effects of radiation ie. skin burns, pathologicfractures, and damages to growth plates were noted to occur. Currently, several largeseries have shown that 5 year survival rates of 75-80% can be achieved withpreoperative chemotherapy to shrink the lesion followed by wide excision of the mass.Occasionally, post-operative radiation may be performed, but the indications are stillunclear. Thus, answers 1, 2, and 3 are incomplete, answer 4 is an old therapeuticregimen, and answer 5 is the most correct answer for the question.
    • Question 42 -Which of the following conditions has the highest rate of malignant change? 1. Ollier’s disease 2. Enchondromatosis 3. Maffucci’s syndrome 4. Multiple exostoses 5. Solitary osteochondroma
    • Answer: 3 Enchondromatosis is known as Ollier’s disease, which carries a 25% chance ofmalignant transformation most commonly chondrosarcoma. A solitary exostosis carriesa less than 1% chance of malignant transformation while multiple can be up to 25%.Maffucci’s syndrome is by definition multiple enchondromas with hemangiomas has anear 100% chance of malignant transformation, therefore is the correct response.
    • OITE 1997Question 1 -A 55-year-old woman reports a spontaneous onset of severe pain in her ribs. AP andlateral chest radiographs show severe osteopenia, two rib fractures, and three vertebralcompression fractures. Laboratory studies show a hemoglobin level of 9.0g/dL and amonoclonal spike on serum protein electrophoresis. Which of the following imagingstudies would be most helpful in establishing the diagnosis? 1. Skeletal survey 2. Technical bone scan 3. Bone density determination 4. MRI scan of the thoracic spine 5. CT scan of the chest and abdomen
    • Answer: 1 The inital presentation of this patient suggests either mutiple myeloma (MM) ormetastatic CA (insidious pain onset, fractures and fracture locations). The laboratory shows ananemia which can be consistent with both MM and metastatic CA (but is found in 62% of MMpatients). The monoclonal spike on serum electrophoresis according to RA Kyle ("MultipleMyeloma: Review of 869 Cases"), is present in 76% of MM patients. Kyle also found 79% ofMM patients show skeletal radiographic abnormalities. The characteristic radiographic lesions of MM are punched-out lytic areas withoutosteoblastic changes, which may be sharply circumscribed. The vertebrae, skull, thoracic cage,pelvis, and proximal portions of humerus and femur are the most common sites of involvement ofMM. The vertebral pedicles are rarely involved in myeloma, whereas they are frequently the siteof metastatic CA. "In our [the authors] experience, diphosphonate bone scans are inferior to conventionalroentgenography (for MM)". "...in cases of carcinoma... the scan is superior". "Multiple osseouslesions will be demonstrated on the radiographic skeletal survey in 90% of patients... and willhelp determine [biopsy sites] (to diagnose MM vs. metastatic CA) " . "...radionuclide scans areusually considerably more sensitive than conventional radiographs for the early detection ofskeletal metastases," according to Sim and Frassica. ("Metastatic Bone Disease and Myeloma" inEvarts, CM: Surgery of the Musculoskeletal System, ed. 2 p. 5022). Since the preliminary diagnosis favors MM, the better response is skeletalsurvey to determine biopsy sites and not the bone scan.
    • Question 3- What is the most common metastatic carcinoma to the hand? 1. Lung 2. Renal 3. Breast 4. Thyroid 5. Prostate
    • Answer: 1 Kann, SE, et al (in Instructional Course Lectures 46) note "40% of metastasesto the hand (55 of 129 in four series) arose from a primary lung tumor. The other site ofprimary lesions are the breast, kidney, colon, thyroid, head and neck, and prostate."
    • Question 4An otherwise healthy 45-year old man has an intraosseous low-grade chondrosarcomaof the distal femur with no dedifferentiation or metastatic disease. Treatment shouldconsist of 1. surgical resection only. 2. radiation therapy only. 3. radiation therapy and surgical resection. 4. chemotherapy only. 5. chemotherapy and surgical resection.
    • Answer: 1 "The rate of DNA synthesis in even high-grade [chondrosarcomas] is verylow...Thus, radiation or chemotherapeutic agents that destroy primary tumors byinterfering with DNA synthesis would be predicted to have limited effect." (Mankin etal "Chondrosarcoma of Bone" in Evarts, CM: Surgery of the Musculoskeletal System,ed. 2) Bauer, et al, (in Acta Orthop. Scand. 1995; 66(3): 283-288) analyzed 40 lowgrade chondrosarcomas of the extremities. Their ten year local recurrence rate was 0.09and no metastases were found in 23 patients treated with intralesional curettage alone.The authors feel their results imply treatment with limited surgery would be beneficialcompared with the morbidity associated with en bloc resection and reconstruction.
    • OITE 2006--Ortho Diseases Question 114 Which of the following tumors has the greatest potential to metastasize to the lung?• 1- Osteoblastoma• 2- Enostosis• 3- Desmoplastic fibroma• 4- Giant cell tumor• 5- Enchondroma
    • OITE 2006--Ortho Diseases Question 114 Which of the following tumors has the greatest potential to metastasize to the lung?• 1- Osteoblastoma• 2- Enostosis• 3- Desmoplastic fibroma• 4- Giant cell tumor• 5- Enchondroma
    • 42. Which of the following bone tumors containsepithelial cells in addition to mesenchymal cells?1. Osteosarcoma2. Adamantinoma3. Conventional chondrosarcoma4. Osteoma5. Giant cell tumor
    • 42. Which of the following bone tumors containsepithelial cells in addition to mesenchymal cells?1. Osteosarcoma2. Adamantinoma3. Conventional chondrosarcoma4. Osteoma5. Giant cell tumor
    • Adamantinoma • Pathology: Basic histology consists of nests of epithelial or epitheliod cells surrounded by a fibrous stroma (see image). The epithelial component can take several different patterns (nests, islands, cords, or tubules). The most common pattern is a basaloid pattern – nests of epithelial cells with peripheral palisading. The epithelial cells are positive for cytokeratin, while the stromal cells are postitive for vimentin.Desai SS, Jambhekar N, Agarwal M, et al: Adamantinoma oftibia: A study of 12 cases. J Surg Oncol 2006;93:429-433.
    • 89) Increased telemorase activity in chondrosarcoma, as determined by immunohistochemistry, has been shown to directly correlate with1) Decreased tumor grade2) The rate of recurrence3) Technetium activity on bone scan4) Gadolinium uptake on MRI5) Tumor senescence.
    • 1) Decreased tumor grade2) The rate of recurrence3) Technetium activity on bone scan4) Gadolinium uptake on MRI5) Tumor senescence
    • Martin JA, DeYoung BR, Gitelis S, et al. Telemorase reverse transcriptase subunit expression is associated with chondrosarcoma malignancy. Clin Orthop Rleat Res 2004; 426: 117-124.-cartilage neoplasias include malignant chondrosarcomas and benign enchondromas. Aggressive chondrosarcomas are often difficult to distinguish from less aggressive ones, or benign enchondromas. They all express aggrecan rich type II collagen reinforced ECM that resembles normal cartilage architecture. Therefore the need to develop malignancy specific markers for these tumors.
    • 57. A 58 yo man has had increasing stiffness and swellingin the right knee for the past 9 months. Radiographs anda biopsy specimen are shown in figures 19A-C. What isthe most likely diagnosis?1. Tumoral Calcinosis2. Synovial Chondromatosis3. Synovial Hemangioma4. Synovial Sarcoma5. Periosteal Osetosarcoma
    • 57. The answer is B. Synovial Chondromatosis.The XR shows a knee with significant nodularcartilage and bone formation.The biopsy clearly shows balls of chondrocytescoated in synovial lining cells.Patients usually present as this patient did with astiff joint with swelling, usually with mechanicalsymptoms.Tx: Synovectomy + removal of loose bodies.
    • 63. An 11 yo girl has knee pain and a limp. A radiographand an MRI are shown in figures 20A-B respectively. Abiopsy specimen is shown in Figure 20C. Routinelaboratory testing will most likely show an elevation in whichof the following?1 – AlkalinePhosphatase2 – Total Bilirubin3 – Hematocrit4 – Lymphocytes5 – Alpha Fetal Protein
    • 63. The answer is 1 - alkaline phosphatase.The image shows an osteogenic sarcoma. Due tosignificant bony turnover, ALK PHOS will beelevated.* Total Bilirubin – elevated with involvement ofhepatobiliary system.•Hematocrit – can be elevated during hematologiccancers.•Lymphocytes – elevated with infection/hemeonc•AFP – elevated with germ cell tumors
    • 121. Figure 37 shows the radiograph of a 15 yoboy who has had knee pain for the past 3months. What is the next most appropriate stepin management?1 – Close observation with a follow upradiograph in 6 weeks2 – Biopsy3 – CT scan of the chest, a bone scan, and aMRI of the femur4 – Cultures and IV antibiotics5 – Radiofrequency ablation
    • 121. The answer is C. CT chest, bone scan,and MRI of the femur.This boy has a lesion concerning for malignancy based on sizeof tumor, symptoms of continued pain, and effect of lesion onbone.•Standard staging w/u includes: Plain XR of limb CT/MRI entire bone: eval compartments/bone CXR/CT Chest: Eval for pulmonary mets Bone Scans: Eval for other bony lesions Lab tests: tailored for eval of other system involvement including LFTs, CBC/diff, ESR/CRP, Ca/Phos,Serum & Urine Electrophoresis, UA, peripheral bloodsmear, possible thyroid tests/mammagrams, poss CT abdomen/renal US, PSA when searching for primary
    • OITE 2007 #186• A 36-year-old woman has had knee pain for the past 4 months. Radiographs, MRI scans, and a biopsy specimen are shown in Figures 71a through 71e. What is the most likely diagnosis? – 1 Giant cell tumor of bone – 2 Pigmented villonodular synovitis – 3 Chondroblastoma – 4 Rheumatoid Nodule – 5 Osteomyelitis
    • OITE 2007 #186• Figures 71a through 71e
    • OITE 2007 #186• A 36-year-old woman has had knee pain for the past 4 months. Radiographs, MRI scans, and a biopsy specimen are shown in Figures 71a through 71e. What is the most likely diagnosis? – 1 Giant cell tumor of bone – 2 Pigmented villonodular synovitis – 3 Chondroblastoma – 4 Rheumatoid Nodule – 5 Osteomyelitis
    • OITE 2007 #197• Figure 74a and 74b show the radiograph and MRI scans of a 27-year-old woman who has had a 2-moth history of progressive left knee pain. An open biopsy yields the specimen shown in Figures 74c. What is the most appropriate treatment? – 1 Chemotherapy followed by proximal tibia resection with prosthetic reconstruction – 2 Proximal tibia resection with prosthetic reconstruction – 3 Observation – 4 External beam radiation therapy – 5 Intralesional curettage with local adjuvant
    • OITE 2007 #197• Figure 74a, 74b & 74c
    • OITE 2007 #197• Figure 74a and 74b show the radiograph and MRI scans of a 27-year-old woman who has had a 2-moth history of progressive left knee pain. An open biopsy yields the specimen shown in Figures 74c. What is the most appropriate treatment? – 1 Chemotherapy followed by proximal tibia resection with prosthetic reconstruction – 2 Proximal tibia resection with prosthetic reconstruction – 3 Observation – 4 External beam radiation therapy – 5 Intralesional curettage with local adjuvant
    • OITE 2007 #203• A 60-year-old woman has an enlarging painless mass in the upper thigh. A radiograph and T1 and T2 weight MRI scans are shown in Figures 75a – c. A biopsy specimen is in 75d. Staging studies do no reveal any evidence of metastatic disease. What is the next most appropriate step in management? – 1 Chemotherapy – 2 Radiation Therapy – 3 Mammography – 4 Embolization – 5 Surgery
    • OITE 2007 #203• Figures 75a – d
    • OITE 2007 #203• A 60-year-old woman has an enlarging painless mass in the upper thigh. A radiograph and T1 and T2 weight MRI scans are shown in Figures 75a – c. A biopsy specimen is in 75d. Staging studies do no reveal any evidence of metastatic disease. What is the next most appropriate step in management? – 1 Chemotherapy – 2 Radiation Therapy – 3 Mammography – 4 Embolization – 5 Surgery
    • OITE 2007 #203• Chondrosarcoma is the second most common primary malignant bone tumor• Pathology large atypical chondrocytes• ―Although patients who have a high-grade lesion are considered to be at high risk for metastasis, chondrosarcomas generally are treated with operative resection. Adjuvant radiation and chemotherapy have been reserved for patients who have a mesenchymal chondrosarcoma or a dedifferentiated chondrosarcoma or for those who have had inadequate operative treatment.‖• Lee FY. Mankin HJ. Fondren G. Gebhardt MC. Springfield DS. Rosenberg AE. Jennings LC. Chondrosarcoma of bone: an assessment of outcome. JBJS AM. 81(3):326-38, 1999 Mar.• Rex A. W. Marco, Steven Gitelis, Gregory T. Brebach, and John H. Healey. Cartilage Tumors: Evaluation and Treatment JAAOS, September/October 2000; 8: 292 - 304.
    • OITE 2007 #208• A 62-year-old man with a history of nonmetastatic thyroid carcinoma has right hip pain. Radiographs and a CT scan are shown in figure 76a – c. He undergoes placement of a locked IM rod to prevent pathologic fracture. A biopsy specimen is shown in Figure 76d. Staging reveals that this is an isolated lesion. Definitive management should definitely include which of the following? – 1 Iodine 121 – 2 Radiation therapy to the right femur – 3 Physical therapy for ROM and WBAT – 4 Brachytherapy to the right proximal femur – 5 Wide Resection
    • OITE 2007 #208• 76a – d
    • OITE 2007 #208• A 62-year-old man with a history of nonmetastatic thyroid carcinoma has right hip pain. Radiographs and a CT scan are shown in figure 76a – c. He undergoes placement of a locked IM rod to prevent pathologic fracture. A biopsy specimen is shown in Figure 76d. Staging reveals that this is an isolated lesion. Definitive management should definitely include which of the following? – 1 Iodine 121 – 2 Radiation therapy to the right femur – 3 Physical therapy for ROM and WBAT – 4 Brachytherapy to the right proximal femur – 5 Wide Resection
    • 231.A 15-year-old boy reports pain in his posterior thigh. Radiographs and a CT scan are shown in Figures 87a through 87c. A biopsy specimen is shown in Figure 87d. What is the most likely diagnosis? 1 – osteomyelitis 2 – aneurysmal bone cyst 3 – fibrous dysplasia 4 – nonossifying fibroma 5 – malignant fibrous histiocytoma
    • 231.A 15-year-old boy reports pain in his posterior thigh. Radiographs and a CT scan are shown in Figures 87a through 87c. A biopsy specimen is shown in Figure 87d. What is the most likely diagnosis? 1 – osteomyelitis 2 – aneurysmal bone cyst 3 – fibrous dysplasia 4 – nonossifying fibroma 5 – malignant fibrous histiocytoma
    • 242. A 55-year-old man has an isolated femoral fracture following a motor vehicle accident. A radiograph is shown in Figure 91. He is hemodynamically stable and his pain is adequately controlled. What is the next most apporpriate step in management?1 – antegrade intramedullary nailing of femur2 – retrograde intramedullary nailing of the femur3 – placement of a distal femoral traction pin4 – wide resection5 – biospy and staging
    • 242. A 55-year-old man has an isolated femoral fracture following a motor vehicle accident. A radiograph is shown in Figure 91. He is hemodynamically stable and his pain is adequately controlled. What is the next most apporpriate step in management?1 – antegrade intramedullary nailing of femur2 – retrograde intramedullary nailing of the femur3 – placement of a distal femoral traction pin4 – wide resection5 – biospy and staging
    • 254.A 48-year-old man reports a mass in his thigh. He denies any history of significant trauma. MRI scans are shown in Figures 96a through 96c. What is the most likely diagnosis?1 – round cell liposarcoma2 – dedifferentiated liposarcoma3 – synovial sarcoma4 – intramuscular lipoma5 – hematoma
    • 254.A 48-year-old man reports a mass in his thigh. He denies any history of significant trauma. MRI scans are shown in Figures 96a through 96c. What is the most likely diagnosis?1 – round cell liposarcoma2 – dedifferentiated liposarcoma3 – synovial sarcoma4 – intramuscular lipoma5 – hematoma
    • 273.Chemotherapy has been shown to improve survival for all of the following malignancies EXCEPT1 – osteosarcoma2 – Ewing’s sarcoma3 – chondrosarcoma4 – lymphoma of bone5 – rhabdomyosarcoma
    • 273.Chemotherapy has been shown to improve survival for all of the following malignancies EXCEPT1 – osteosarcoma2 – Ewing’s sarcoma3 – chondrosarcoma4 – lymphoma of bone5 – rhabdomyosarcoma
    • OITE 2006 – Ortho Diseases7. A 12-year-old patient has an epiphyseal lesion. What is the most likely diagnosis? 1- Giant cell tumor 2- Aneurysmal bone cyst 3- Eosinophilic granuloma 4- Dysplasia epiphysealis hemimelica 5- Chondroblastoma
    • OITE 2006 – Ortho Diseases7. A 12-year-old patient has an epiphyseal lesion. What is the most likely diagnosis? 1- Giant cell tumor 2- Aneurysmal bone cyst 3- Eosinophilic granuloma 4- Dysplasia epiphysealis hemimelica 5- Chondroblastoma
    • OITE 2006 – Ortho Diseases23. An 80-year-old woman reports the sudden development of pain in the left distal thigh. She denies any history of trauma. Figures 9a through 9d show radiographs, a bone scan, and a biopsy specimen. What is the likely diagnosis? 1- Enchondroma 2- Osteosarcoma 3- Dedifferentiated chondrosarcoma 4- Metastatic breast carcinoma 5- Paget’s disease
    • OITE 2006 – Ortho Diseases
    • OITE 2006 – Ortho Diseases
    • OITE 2006 – Ortho Diseases23. An 80-year-old woman reports the sudden development of pain in the left distal thigh. She denies any history of trauma. Figures 9a through 9d show radiographs, a bone scan, and a biopsy specimen. What is the likely diagnosis? 1- Enchondroma 2- Osteosarcoma 3- Dedifferentiated chondrosarcoma 4- Metastatic breast carcinoma 5- Paget’s disease
    • OITE 2006 – Ortho Diseases23. An 80-year-old woman reports the sudden development of pain in the left distal thigh. She denies any history of trauma. Figures 9a through 9d show radiographs, a bone scan, and a biopsy specimen. What is the likely diagnosis? 1- Enchondroma 2- Osteosarcoma 3- Dedifferentiated chondrosarcoma 4- Metastatic breast carcinoma 5- Paget’s disease
    • OITE 2006 Orthopedic Diseases78. A 14 year old girl reports right thigh discomfort with activities. An AP radiograph is shown in figure 24a, and coronal T1-weighted and axial T2-weighted MRI scans are shown if figures 24b and 24c, respectively. A biopsy specimen is shown in Figure 24d. What is the treatment of choice for this tumor? 1- Surgery only 2- Surgery and chemotherapy 3- Surgery and radiation therapy 4- Chemotherapy only 5- Chemotherapy and radiation therapy
    • OITE 2006 Orthopedic Diseases
    • OITE 2006 Orthopedic Diseases78. A 14 year old girl reports right thigh discomfort with activities. An AP radiograph is shown in figure 24a, and coronal T1-weighted and axial T2-weighted MRI scans are shown if figures 24b and 24c, respectively. A biopsy specimen is shown in Figure 24d. What is the treatment of choice for this tumor? 1- Surgery only 2- Surgery and chemotherapy 3- Surgery and radiation therapy 4- Chemotherapy only 5- Chemotherapy and radiation therapy
    • OITE 2006 Orthopedic Diseases79. A 25 year old man reports progressive wrist pain and swelling. An AP radiograph and biopsy specimen are shown in Figures 25a and 25b. Management should consist of: 1- below-elbow amputation 2- bisphosphonate treatment 3- nonsteroidal anti-inflammatory drugs 4- chemotherapy followed by wide surgical excision 5- intralesional resection and reconstruction
    • OITE 2006 Orthopedic Diseases
    • OITE 2006 Orthopedic Diseases79. A 25 year old man reports progressive wrist pain and swelling. An AP radiograph and biopsy specimen are shown in Figures 25a and 25b. Management should consist of: 1- below-elbow amputation 2- bisphosphonate treatment 3- nonsteroidal anti-inflammatory drugs 4- chemotherapy followed by wide surgical excision 5- intralesional resection and reconstruction
    • OITE 2006 Orthopedic Diseases84. A 57 year old man was diagnosed with localized prostate carcinoma 3 years ago, with negative margins and negative lymph nodes. He now reports a 3-week history of severe right hip pain that is worse with weight-bearing. Radiographs are shown in Figures 26a and 26b. CT scans of the chest, abdomen, and pelvis, as well as a whole body bone scan, are negative for other lesions. What is the next most appropriate step for treatment? 1- Right hip hemiarthroplasty 2- Locked IM rod placement in right femur 3- Biopsy of the right femur 4- Hip disarticulation 5- Curettage, cementation, and plate fixation
    • OITE 2006 Orthopedic Diseases
    • OITE 2006 Orthopedic Diseases84. A 57 year old man was diagnosed with localized prostate carcinoma 3 years ago, with negative margins and negative lymph nodes. He now reports a 3-week history of severe right hip pain that is worse with weight-bearing. Radiographs are shown in Figures 26a and 26b. CT scans of the chest, abdomen, and pelvis, as well as a whole body bone scan, are negative for other lesions. What is the next most appropriate step for treatment? 1- Right hip hemiarthroplasty 2- Locked IM rod placement in right femur 3- Biopsy of the right femur 4- Hip disarticulation 5- Curettage, cementation, and plate fixation
    • OITE 2006 Orthopedic Diseases96. A 12 year old sustained a knee injury in a fall off his bike. The patient reports that his knee pain improved the day after injury. Radiographs shown in Figures 30a and 30b reveal a lesion in the distal femur. Management should consist of 1- open biopsy and intralesional excision 2- CT-guided needle biopsy and anti-microbial therapy 3- curettage and autogenous iliac crest bone grafting 4- anti-inflammatory drugs as needed 5- observation and follow up
    • OITE 2006 Orthopedic Diseases
    • OITE 2006 Orthopedic Diseases96. A 12 year old sustained a knee injury in a fall off his bike. The patient reports that his knee pain improved the day after injury. Radiographs shown in Figures 30a and 30b reveal a lesion in the distal femur. Management should consist of 1- open biopsy and intralesional excision 2- CT-guided needle biopsy and anti-microbial therapy 3- curettage and autogenous iliac crest bone grafting 4- anti-inflammatory drugs as needed 5- observation and follow up
    • OITE 2006 Orthopedic Diseases109. A 72 year old man with a history of smoking 40 packs of cigarettes per year underwent a successful left total hip replacement 10 years ago. He now reports a 2 month history of progressive right hip pain. An AP pelvic radiograph and CT scan are shown in Figures 35a and 35b. What is the next most appropriate step in management? 1- Immediate cemented right total hip arthroplasty 2- Open reduction and internal fixation of the acetabular fracture 3- Activity modification, IV bisphosphonates, and follow up in 6 weeks 4- Technetium Tc 99m scan and CT of the chest, abdomen, and pelvis 5- Radiation therapy
    • OITE 2006 Orthopedic Diseases
    • OITE 2006 Orthopedic Diseases109. A 72 year old man with a history of smoking 40 packs of cigarettes per year underwent a successful left total hip replacement 10 years ago. He now reports a 2 month history of progressive right hip pain. An AP pelvic radiograph and CT scan are shown in Figures 35a and 35b. What is the next most appropriate step in management? 1- Immediate cemented right total hip arthroplasty 2- Open reduction and internal fixation of the acetabular fracture 3- Activity modification, IV bisphosphonates, and follow up in 6 weeks 4- Technetium Tc 99m scan and CT of the chest, abdomen, and pelvis 5- Radiation therapy
    • OITE 2006--Ortho Diseases Question 119: A 25 year-old man has had intermittent swelling in the left thenar eminence for the past few months. He describes the onset of a dull ache associated with swelling. An AP radiograph is shown in Figure 39. What is the most likely diagnosis?• 1- Lipoma• 2- Hemangioma• 3- Synovial sarcoma• 4- Ewing’s family of tumor• 5- Giant cell tumor of the tendon sheath
    • OITE 2006--Ortho DiseasesQuestion 119: Figure 39
    • OITE 2006--Ortho Diseases Question 119: A 25 year-old man has had intermittent swelling in the left thenar eminence for the past few months. He describes the onset of a dull ache associated with swelling. An AP radiograph is shown in Figure 39. What is the most likely diagnosis?• 1- Lipoma• 2- Hemangioma• 3- Synovial sarcoma• 4- Ewing’s family of tumor• 5- Giant cell tumor of the tendon sheath
    • OITE 2006--Ortho Diseases Question 137 Which of the following diseases of bone (when nonmetastatic at diagnosis) carries the worst prognosis for 5-year survival)?• 1- Lymphoma• 2- Osteosarcoma• 3- Ewing’s sarcoma• 4- Paget’s sarcoma• 5- Conventional chondrosarcoma
    • OITE 2006--Ortho Diseases Question 137 Which of the following diseases of bone (when nonmetastatic at diagnosis) carries the worst prognosis for 5-year survival)?• 1- Lymphoma• 2- Osteosarcoma• 3- Ewing’s sarcoma• 4- Paget’s sarcoma• 5- Conventional chondrosarcoma
    • OITE 2006--Ortho Diseases Question 147: A 2-year-old boy has a limp and anterior bowing of the leg. A lateral radiograph is shown in Figure 52. What is the most likely diagnosis?• 1- Unicameral bone cyst• 2- Osteofibrous dysplasia• 3- Adamantinoma• 4- Ewing’s sarcoma• 5- Metastatic neuroblastoma
    • OITE 2006--Ortho DiseasesQuestion 147: Figure 52
    • OITE 2006--Ortho Diseases Question 147: A 2-year-old boy has a limp and anterior bowing of the leg. A lateral radiograph is shown in Figure 52. What is the most likely diagnosis?• 1- Unicameral bone cyst• 2- Osteofibrous dysplasia• 3- Adamantinoma• 4- Ewing’s sarcoma• 5- Metastatic neuroblastoma
    • OITE 2006--Ortho Diseases Question 161: A 16 year-old girl has had vague right hip pain for the past 2 months. An AP radiograph of the pelvis and a T1 fat suppression, gadolinium-enhanced MRI are shown in Figures 62a and 62b. A biopsy specimen is shown in Figure 62c. What characteristic genetic translocation is associated with this disease?• 1- t(X;18)• 2- t(2;13)• 3- t(9;22)• 4- t(11;22)• 5- t(12;16)
    • OITE 2006--Ortho DiseasesQuestion 161: Figure 62a
    • OITE 2006--Ortho DiseasesQuestion 161: Figure 62b
    • OITE 2006--Ortho DiseasesQuestion 161: Figure 62c
    • OITE 2006--Ortho Diseases Question 161: A 16 year-old girl has had vague right hip pain for the past 2 months. An AP radiograph of the pelvis and a T1 fat suppression, gadolinium-enhanced MRI are shown in Figures 62a and 62b. A biopsy specimen is shown in Figure 62c. What characteristic genetic translocation is associated with this disease?• 1- t(X;18)• 2- t(2;13)• 3- t(9;22)• 4- t(11;22)• 5- t(12;16)
    • Question 16Figure 4a shows a pigmented lesion on the right side of the neck of a 41-year-old man.The patients history reveals that he had multiple bone lesions during childhood andjuvenile-onset diabetes mellitus. Figure 4b and 4c show radiographs of his knee andleg. What is the most likely diagnosis? 1. Ollier’s disease 2. Neurofibromatosis 3. McCune-Albright syndrome 4. Multiple hereditary exostoses 5. Multiple nonossifying fibromas
    • Answer: 3The classic triad seen with McCune-Albright Syndrome is precocious puberty,polyostotic fibrous dysplasia (seen in fig 4B and 4C) and café au lait spots (seen in fig4A). The fibrous dysplasia is metaphyseal or diaphyseal and associated bowing iscommon. Olliers disease is associated with multiple enchondromatoses, but does notinclude café au lait spots. Neurofibromatosis is associated with café au lait spots, butthey are generally on the trunk, pelvis and flexor creases of elbows and knees. Multiplecutaneous fleshy tumors are also frequently seen by late childhood.Source:Campbells Operative Orthopedics Atlas of Orthopedic Pathology Merck Manual
    • Question 23 -A 10-year old boy has had intermittent pain in his right groin and proximal thigh for thepast 6 months. Fingures 6a and 6b show plain radographs of the hip. Figure 6c showsan axial proton density MRI scan through the lesion, and Figure 6d showsrepresentative tissue biopsy specimens at low power. What is the most likely diagnosis? 1. Chondroblastoma 2. Ewing’s sarcoma 3. Giant cell tumor 4. Simple bone cyst 5. Aneurysmal bone cyst
    • Answer: 5Clinical Symptoms- pain is short in durationB. Radiographic Features- ballooned or cystic expansion of the bone no significant matrix the lesion affects the metaphysis of long bones no significant mineralizationMRI- well defined lesion often with lobulated contour and internal septation withmultiple fluid levels.C. Histology- hemosiderin laden macrophages, multinucleated giant cells, fibrous stroma, and woven bone.Kransdorf MS., and Berquist TH: Musculoskeletal Neoplasms; MRI of TheMusculoskeletal System 1996. pp 822-836.Springfield DS: Bone and Soft Tissue Tumors; Lovell and Winter’s PediatricOrthpaedics 1996. pp50-51.Wold LE., McLeod RA., Sim FH., and Unni KK: Atlas Of Orthopedic Pathology 1990.pp232-237
    • Question 67A 12-year-old girl has had painful, unilateral toe walking for the past 12 months.Examination shows that her foot is fixed in equinus, and she has exquisite pointtenderness over the proximal and medial aspect of the medial gastrocnemius muscle. Alateral radiograph of the knee is shown in figure 16a, and a T2-weighted axial MRIscan of the proximal leg is shown in figure 16b. A photomicrograph of biopsymaterial is shown in Figure 16c. What is the most likely diagnosis? 1. Rhabdomyosarcoma 2. Nodular Fascitis 3. Heterotopic Ossification 4. Soft-tissue hemangioma 5. Soft-tissue Ewing’s sarcoma
    • Answer: 4Soft tissue hemangioma are common and many types are present at birth. Can occur deep inskeletal muscle and other soft tissue of the extremities. Tend to be bluish in color. Cavernoustypes frequently are visible on plain x-rays because of areas of calcifications that can bedescribed as smoke rings. These are felt to be pathognomonic (Enneking). Biopsy showsvascular components that may difficult to tell from normal vascular tissues. MRI shows aheterogeneous lesion with numerous small blood vessels and fatty infiltrate. Nonoperativemeasures are used if can control discomfort. Operative treatment is wide resection, howeverlocal recurrence is high (Miller). Rhabdomyosarcoma is a malignant tumor of skeletal muscle,and is more common in children and adolescents. Also involved in high rate of lymph nodemetastasis. Treatment includes wide resection, radiotherapy and prolonged systemicpolychemotherapy. Nodular fascitis consists of proliferating fibroblasts in myoid stroma withpredominant vascular pattern. Occurs most frequently in forearm of young adults with deepfascia involved. Heterotopic ossification is usually from repetitive trauma-most commonly overdiaphyseal long bones. Usually not attached to underlying bone. There is a zonal pattern withtrabecular bone at peripherally and immature tissue at center. Ewing’s is rapid growing painfulmass that tender on exam. Usually begins in central portion of bone and rapidly extendsproximal and distal along canal and through vascular perforations. Bone is rapidly resorbed andas characteristic periosteal reaction (Codman’s triangle). (Enneking, Miller)Enzinger F, Weiss S (eds): Soft Tissue Tumors, ed 3. St Louis, MO, CV Mosby, 1995, pp605-609.
    • Question 80Figures 22a and 22b show plain xrays of a 33-year-old man who has had progressivepain in his nondominant left shoulder for the past 5 months. A proton density MRI isshown in Fig. 22c, and histiologic materials from the solid portion of the lesion areshown in Figures 22d and 22e. What is the most likely diagnosis? 1. Enchondroma 2. Giant cell tumor 3. Chondroblastoma 4. Chondromyxoid fibroma 5. Clear cell chondrosarcoma
    • Answer: 5Ref.: Unni KK; Dahlin’s Bone Tumors 1996, pp. 71-108-males 70%, females 30%-peak age; third decade-most common location: the proximal femur accounts for approx. 50%. nearly always involves the epiphyseal region of a long bone.-clinical symptoms: pain is the most common at presentation-X-ray fractures: lesion has epiphyseal location in prox. femur or humerus
    • Question 165 -Figures 44a and 44b show the plain radiographs of a 12-year-old boy who has had leftmedial knee pain for the past 4 months. Figure 44c shows representative histologicmaterial. What is the most likely diagnosis? 1. enchondroma 2. osteoblastoma 3. giant cell tumor 4. chondroblastoma 5. osteochondritis dessicans
    • Answer: 4 Chondroblastoma is a lytic lesion commonly located eccentrically in theepiphysis of long bones, most often in skeletally immature individuals. The mostcommon sites of origin are the proximal humerus, proximal and distal femur, andproximal tibia. The radiographic differential includes giant cell tumor crossing into theepiphysis. In this case however, the characteristic highly cellular chondral ovalmononuclear cells seen on the histologic material confirm the diagnosis.Turcotte RE, Kurt AM, Sim FH, et al. Chondroblastoma. Human Pathology 1993;24: 944-949.
    • Question 219 -A 35-year old man has had increasing pain in the knee for the past 4 months. An APradiograph of the knee is shown in Figure 58a, and low- and high-powerphotomicrographs of the biopsy specimen are shown in Figures 58b and 58c. What isthe most likely diagnosis? 1. Osteosarcoma 2. Chondroblastoma 3. Giant Cell tumor 4. Aneurysmal bone cyst 5. Desmoplastic fibroma
    • Answer: 350% of Giant cell tumors usually occur about the knee, with 28% involving distalfemur. It is a lytic lesion with periosteal reaction. Histologically giant cells scattereduniformly, and the nuclei of the mononuclear and giant cells are similar in appearance.Most common age is 3rd decade. In this case the radiographic finding are similar toosteosarcoma and Desmoplastic fibroma as well as Giant cell tumor, however,histologic study singles out Giant cell tumor.(Atlas of orthopedic surgery)
    • Question 222 -Figures 60a and 60b show the radiographs of the ankle and distal leg of an 11-year-oldgirl after she twisted her ankle while playing soccer. She has no history of ankle or legpain. Examination reveals localized swelling and tenderness over the lateral ankle, andthe tibia is not tender. The bone lesion identified in the tibia most likely is 1. osteoblastoma 2. osteoid osteoma 3. ossifying fibroma 4. fibrous dysplasia 5. nonossifying fibroma
    • Answer: 5DISCUSSION: Nonossifying fibroma almost always develops in patients under theage of twenty and is usually discovered when a x-ray is taken for an injury.Histologically, there is a triphasic cytological appearance, featuring oval round cells,spindle cells, and giant cells. It usually is a single well-delineated cortical defect withcircular or oval lesions and smooth, lobulated edges. Adjacent body is sclerotic withoutperiosteal reaction. The lower extremity long tubular bones are the most common sitesof occurrence. Treatment is usually not necessary, but occasionally a large lesion willrequire intralesional excision and bone grafting.REFERENCES: Marks KE, Bauer TW: Fibrous Tumors of Bone. Orthop Clin NorthAm 1989; 20(3):377-93.
    • Question 227An 80 year-old man has had increasing hip pain and difficulty ambulating for the past 6months. An oblique radiograph pf the hip is shown in figure 64a, and a technetium bonescan is shown in Figure 64b. Low-and high power photomicrographs are shown inFigure 64c and 64d. What is the most likely diagnosis? 1. Pagets sarcoma 2. Pagets disease 3. Fibrous dysplasia 4. Hyperparathyroidism 5. Metastatic carcinoma
    • Answer: 2Pagets disease is generally discovered in the patient 50 years of age and older. Thisdisease in more common in those of eastern and western European decent and rare inAsians, Scandinavians, and blacks. 20% of patients are asymptomatic and the diagnosisis made while being worked-up for an unrelated complaint. Symptomatic patientscomplain of bone pain, skeletal deformities, changes in skin temperature, pathologicfractures and symptoms related to nerve compression. Radiographic appearance is thatof focal bone resorption and formation in a disordered trabecular pattern, with enlargedcortices and overall bone size. Pagets disease appears hot on technetium bone scans.Treatment is indicated for those with increasing pain and/or deformity. Calcitonin anddiphosphonates are the pharmacologic agents available. Pathologic fractures, disablingarthritis, and severe malalignment are indications for surgical intervention.REFERENCES: Merkow RL, Lane JM: Pagets Disease of Bone. Orthop Clinics NorthAm 1990;21(1):171-89.
    • OITE 2008 – Basic Science / Onc• 8 Figure 3a shows the radiograph of a 30-year-old woman who reports worsening left hip pain. Bone and CT scans do not reveal any other sites of disease. A biopsy specimen is shown in Figure 3b. The immunostaining reactivity for smooth muscle actin is shown in Figure 3c. What is the most likely diagnosis?• 1- Ostegenic sarcoma• 2- Ewings sarcoma• 3- Metastatic carcinoma• 4- Malignant fibrous histiocytoma of bone• 5- Leiomyosarcoma of bone
    • OITE 1997Question 1 -A 55-year-old woman reports a spontaneous onset of severe pain in her ribs. AP andlateral chest radiographs show severe osteopenia, two rib fractures, and three vertebralcompression fractures. Laboratory studies show a hemoglobin level of 9.0g/dL and amonoclonal spike on serum protein electrophoresis. Which of the following imagingstudies would be most helpful in establishing the diagnosis? 1. Skeletal survey 2. Technical bone scan 3. Bone density determination 4. MRI scan of the thoracic spine 5. CT scan of the chest and abdomen
    • Answer: 1 The inital presentation of this patient suggests either mutiple myeloma (MM) ormetastatic CA (insidious pain onset, fractures and fracture locations). The laboratory shows ananemia which can be consistent with both MM and metastatic CA (but is found in 62% of MMpatients). The monoclonal spike on serum electrophoresis according to RA Kyle ("MultipleMyeloma: Review of 869 Cases"), is present in 76% of MM patients. Kyle also found 79% ofMM patients show skeletal radiographic abnormalities. The characteristic radiographic lesions of MM are punched-out lytic areas withoutosteoblastic changes, which may be sharply circumscribed. The vertebrae, skull, thoracic cage,pelvis, and proximal portions of humerus and femur are the most common sites of involvement ofMM. The vertebral pedicles are rarely involved in myeloma, whereas they are frequently the siteof metastatic CA. "In our [the authors] experience, diphosphonate bone scans are inferior to conventionalroentgenography (for MM)". "...in cases of carcinoma... the scan is superior". "Multiple osseouslesions will be demonstrated on the radiographic skeletal survey in 90% of patients... and willhelp determine [biopsy sites] (to diagnose MM vs. metastatic CA) " . "...radionuclide scans areusually considerably more sensitive than conventional radiographs for the early detection ofskeletal metastases," according to Sim and Frassica. ("Metastatic Bone Disease and Myeloma" inEvarts, CM: Surgery of the Musculoskeletal System, ed. 2 p. 5022). Since the preliminary diagnosis favors MM, the better response is skeletalsurvey to determine biopsy sites and not the bone scan.
    • Question 3- What is the most common metastatic carcinoma to the hand? 1. Lung 2. Renal 3. Breast 4. Thyroid 5. Prostate
    • Answer: 1 Kann, SE, et al (in Instructional Course Lectures 46) note "40% of metastasesto the hand (55 of 129 in four series) arose from a primary lung tumor. The other site ofprimary lesions are the breast, kidney, colon, thyroid, head and neck, and prostate."
    • Question 4An otherwise healthy 45-year old man has an intraosseous low-grade chondrosarcomaof the distal femur with no dedifferentiation or metastatic disease. Treatment shouldconsist of 1. surgical resection only. 2. radiation therapy only. 3. radiation therapy and surgical resection. 4. chemotherapy only. 5. chemotherapy and surgical resection.
    • Answer: 1 "The rate of DNA synthesis in even high-grade [chondrosarcomas] is verylow...Thus, radiation or chemotherapeutic agents that destroy primary tumors byinterfering with DNA synthesis would be predicted to have limited effect." (Mankin etal "Chondrosarcoma of Bone" in Evarts, CM: Surgery of the Musculoskeletal System,ed. 2) Bauer, et al, (in Acta Orthop. Scand. 1995; 66(3): 283-288) analyzed 40 lowgrade chondrosarcomas of the extremities. Their ten year local recurrence rate was 0.09and no metastases were found in 23 patients treated with intralesional curettage alone.The authors feel their results imply treatment with limited surgery would be beneficialcompared with the morbidity associated with en bloc resection and reconstruction.
    • Question 16Figure 4a shows a pigmented lesion on the right side of the neck of a 41-year-old man.The patients history reveals that he had multiple bone lesions during childhood andjuvenile-onset diabetes mellitus. Figure 4b and 4c show radiographs of his knee andleg. What is the most likely diagnosis? 1. Ollier’s disease 2. Neurofibromatosis 3. McCune-Albright syndrome 4. Multiple hereditary exostoses 5. Multiple nonossifying fibromas
    • Answer: 3The classic triad seen with McCune-Albright Syndrome is precocious puberty,polyostotic fibrous dysplasia (seen in fig 4B and 4C) and café au lait spots (seen in fig4A). The fibrous dysplasia is metaphyseal or diaphyseal and associated bowing iscommon. Olliers disease is associated with multiple enchondromatoses, but does notinclude café au lait spots. Neurofibromatosis is associated with café au lait spots, butthey are generally on the trunk, pelvis and flexor creases of elbows and knees. Multiplecutaneous fleshy tumors are also frequently seen by late childhood.Source:Campbells Operative Orthopedics Atlas of Orthopedic Pathology Merck Manual
    • Question 20A 42-year-old health care professional has had knee pain for the past 2 months. AnMRI scan of the knee reveals a large effusion with loculations and synovial thickening,and results of an open biopsy and culture are consistent with tuberculosis. Sensitivitytests show no resistance to antibiotics. Following debridement and synovectomy,appropriate antibiotic therapy should include 1. rifampin and pyridoxine. 2. rifampin and ethambutol hydrochloride. 3. isoniazid. 4. isoniazid and pyridoxine. 5. isoniazid, rifampin, pyrazinamide, and pyridoxine.
    • Answer: 5Watts and Lifeso, JBJS 1996;78A:288-298. *Current Concepts Review discusses tuberculosis of bone and joints. The casepresented shows classical findings on MRI. Successful medical treatment oftuberculosis requires the prolonged administration of a minimum of three drugs towhich the organisms are susceptible, and at least one of these drugs must bebactericidal.Isoniazid is the most potent bactericidal drug available and is particularly effectiveagainst actively growing organisms. Rifampin and pyrazinamide are the most effectivesterilizing drugs, and they are specifically effective against bacilli that are dormant andundergo periodic bursts of activity. Ethambutol is bacteriostatic. Streptomycin isbactericidal but must be given parenterally. Other drugs useful for multiple-drug-resistant organisms include ethionamide, cycloserine, kanamycin, capreomycin, andparaaminosalicylic acid.The current recommendation for treatment of adults with musculoskeletal tuberculosisis 300mg of isoniazid a day, 600mg of rifampin a day, and 20-30mg/kg of pyrazinamidea day. 10mg of pyridoxine a day is given as prophylaxis against possible isoniazid-induced neuropathy.
    • Question 23 -A 10-year old boy has had intermittent pain in his right groin and proximal thigh for thepast 6 months. Fingures 6a and 6b show plain radographs of the hip. Figure 6c showsan axial proton density MRI scan through the lesion, and Figure 6d showsrepresentative tissue biopsy specimens at low power. What is the most likely diagnosis? 1. Chondroblastoma 2. Ewing’s sarcoma 3. Giant cell tumor 4. Simple bone cyst 5. Aneurysmal bone cyst
    • Answer: 5Clinical Symptoms- pain is short in durationB. Radiographic Features- ballooned or cystic expansion of the bone no significant matrix the lesion affects the metaphysis of long bones no significant mineralizationMRI- well defined lesion often with lobulated contour and internal septation withmultiple fluid levels.C. Histology- hemosiderin laden macrophages, multinucleated giant cells, fibrous stroma, and woven bone.Kransdorf MS., and Berquist TH: Musculoskeletal Neoplasms; MRI of TheMusculoskeletal System 1996. pp 822-836.Springfield DS: Bone and Soft Tissue Tumors; Lovell and Winter’s PediatricOrthpaedics 1996. pp50-51.Wold LE., McLeod RA., Sim FH., and Unni KK: Atlas Of Orthopedic Pathology 1990.pp232-237
    • Question 42 -Which of the following conditions has the highest rate of malignant change? 1. Ollier’s disease 2. Enchondromatosis 3. Maffucci’s syndrome 4. Multiple exostoses 5. Solitary osteochondroma
    • Answer: 3 Enchondromatosis is known as Ollier’s disease, which carries a 25% chance ofmalignant transformation most commonly chondrosarcoma. A solitary exostosis carriesa less than 1% chance of malignant transformation while multiple can be up to 25%.Maffucci’s syndrome is by definition multiple enchondromas with hemangiomas has anear 100% chance of malignant transformation, therefore is the correct response.
    • Question 50 -A 21 year old man has had increasing hip pain for the last 3 months that occursprimarily during weightlifting exercises. AP and oblique radiographs of his hip areshown in figures 13a and 13b. A CT scan of his hip is shown in figure 13c, and a T2-weighted coronal MRI scan is shown in fig.13d. Low and high powerphotomicrographs of the biopsy material are shown in 13e and 13f. What is the mostlikely diagnosis? 1. chondroblastoma 2. giant cell tumor 3. unicameral bone cyst 4. aneurysmal bone cyst 5. hyperparathyroidism
    • Answer: 4Physical exam - pain and swelling are the important features, and they vary in durationfrom weeks to a few years. Lesion tends to increase in size until therapy initiated.X-rays - a zone of rarefaction, which is usually well circumscribed and eccentric, isassociated with an obvious soft-tissue extension. In the classic case, this soft tissueextension is produced by the bulging of periosteum and a resultant layer ofradiographically visible new bone that delimits the periphery of the tumor. The lesionalarea tends to show trabeculation. Fusiform expansion may be produced when smallbones such as ribs or the fibula are affected. They may grow rapidly and mimic amalignancy, especially telangiectatic osteosarcoma. Gross path - the cyst containsanastomosing cavernomatous spaces that ordinarily comprise the bulk of the lesion. Thespaces are usually filled with unclotted blood; the blood may well up into, but not spurtfrom, the tumor when it is unroofed. The eggshell-thick layer of subperiosteal newbone, which delimits the lesion, is ordinarily discernible. With magnification, cavernousspaces that may be filled with blood are identified. The walls of the spaces containspindled fibroblastic cells, multinucleated giant cells, and thin strands of bone.
    • Question 67A 12-year-old girl has had painful, unilateral toe walking for the past 12 months.Examination shows that her foot is fixed in equinus, and she has exquisite pointtenderness over the proximal and medial aspect of the medial gastrocnemius muscle. Alateral radiograph of the knee is shown in figure 16a, and a T2-weighted axial MRIscan of the proximal leg is shown in figure 16b. A photomicrograph of biopsymaterial is shown in Figure 16c. What is the most likely diagnosis? 1. Rhabdomyosarcoma 2. Nodular Fascitis 3. Heterotopic Ossification 4. Soft-tissue hemangioma 5. Soft-tissue Ewing’s sarcoma
    • Answer: 4Soft tissue hemangioma are common and many types are present at birth. Can occur deep inskeletal muscle and other soft tissue of the extremities. Tend to be bluish in color. Cavernoustypes frequently are visible on plain x-rays because of areas of calcifications that can bedescribed as smoke rings. These are felt to be pathognomonic (Enneking). Biopsy showsvascular components that may difficult to tell from normal vascular tissues. MRI shows aheterogeneous lesion with numerous small blood vessels and fatty infiltrate. Nonoperativemeasures are used if can control discomfort. Operative treatment is wide resection, howeverlocal recurrence is high (Miller). Rhabdomyosarcoma is a malignant tumor of skeletal muscle,and is more common in children and adolescents. Also involved in high rate of lymph nodemetastasis. Treatment includes wide resection, radiotherapy and prolonged systemicpolychemotherapy. Nodular fascitis consists of proliferating fibroblasts in myoid stroma withpredominant vascular pattern. Occurs most frequently in forearm of young adults with deepfascia involved. Heterotopic ossification is usually from repetitive trauma-most commonly overdiaphyseal long bones. Usually not attached to underlying bone. There is a zonal pattern withtrabecular bone at peripherally and immature tissue at center. Ewing’s is rapid growing painfulmass that tender on exam. Usually begins in central portion of bone and rapidly extendsproximal and distal along canal and through vascular perforations. Bone is rapidly resorbed andas characteristic periosteal reaction (Codman’s triangle). (Enneking, Miller)Enzinger F, Weiss S (eds): Soft Tissue Tumors, ed 3. St Louis, MO, CV Mosby, 1995, pp605-609.
    • Question 71A 12 year old girl has a Ewing’s sarcoma of the proximal fibula with no metastaticdisease or neurovascular involvment. Treatment should include 1. radiation therapy. 2. chemotherapy. 3. surgical resection. 4. radiation therapy and surgical resection. 5. chemotherapy and surgical resection.
    • Answer: 5 Ewings sarcoma is the second most common primary bone tumor in children.It usually occurs in the first or second decades of life. Radiographically it has a highlyaggressive appearance with permeative bone destruction occurring in the diaphysis oflong bones. It may also occur, however, in flat bones. Periosteal reaction and soft tissuemass without calcifications are common. The periosteal reaction may have a sunburst oronion skin appearance. Radiographic differential diagnosis includes histiocytosis X,osteomyelitis, osteosarcoma, and medullary neuroblastoma. Historically, the 5 year survival rate of the this tumor was found to be about 5-10%. In the past, the use of 5000-6000cGY was utilized to treat the entire bone thatwas affected. This provided good local control of the disease, but it did not preventpulmonary metastasis. Also, the malice effects of radiation ie. skin burns, pathologicfractures, and damages to growth plates were noted to occur. Currently, several largeseries have shown that 5 year survival rates of 75-80% can be achieved withpreoperative chemotherapy to shrink the lesion followed by wide excision of the mass.Occasionally, post-operative radiation may be performed, but the indications are stillunclear. Thus, answers 1, 2, and 3 are incomplete, answer 4 is an old therapeuticregimen, and answer 5 is the most correct answer for the question.
    • Question 80Figures 22a and 22b show plain xrays of a 33-year-old man who has had progressivepain in his nondominant left shoulder for the past 5 months. A proton density MRI isshown in Fig. 22c, and histiologic materials from the solid portion of the lesion areshown in Figures 22d and 22e. What is the most likely diagnosis? 1. Enchondroma 2. Giant cell tumor 3. Chondroblastoma 4. Chondromyxoid fibroma 5. Clear cell chondrosarcoma
    • Answer: 5Ref.: Unni KK; Dahlin’s Bone Tumors 1996, pp. 71-108-males 70%, females 30%-peak age; third decade-most common location: the proximal femur accounts for approx. 50%. nearly always involves the epiphyseal region of a long bone.-clinical symptoms: pain is the most common at presentation-X-ray fractures: lesion has epiphyseal location in prox. femur or humerus
    • Question 89 -A 14-year-old boy has a 4-month history of aching pain in the distal thigh.Examination reveals a mass in the distal thigh. Figure 25a shows a plain radiograph,Figures 25b and 25c show MRI images, Figure 25d shows a bone scan, and Figure 25eshows a CT scan of the chest. The most likely diagnosis and Musculoskelatal TumorSociety (Enneking) stage is 1. osteosarcoma, stage IIB 2. osteosarcoma, stage III 3. parosteal osteosarcoma, stage IIB 4. periosteal osteosarcoma, stage IIB 5. periosteal osteosarcoma, stage III
    • Answer: 2Osteosarcoma commonly occurs about the knee in children and young adults. Patients present primarily withpain. More than 90% of the intramedullary osteosarcomas are high grade and penetrate the cortex early toform a soft tissue mass. About 10-20% of patients have pulmonary metastases at presentation. Plainradiographs demonstrate a lesion in which there is both bone destruction and bone formation. MRI and CTscans are useful for defining the anatomy of the lesion in regard to intramedullary extension, involvement ofneurovascular structures, and muscle invasion.Parosteal Osteosarcoma commonly occurs at the posterior aspect of the distal femur, proximal tibia, andproximal humerus. Patients present with a painless mass. The radiographic appearance is characteristic inthat it demonstrates a heavily ossified, often lobulated mass arising form the cortex.Periosteal Osteosarcoma is a rare form of osteosarcoma and occurs most commonly in the diaphysis of longbones (femur and tibia). The radiographic appearance is fairly constant; a sunburst type lesion rests on asaucerized cortical depression.Staging system of the Musculoskelatal Tumor Society (Enneking System)Stage DescriptionI-A Low grade, intracompartmental, no metastasesI-B Low grade, extracompartmental, no metastasesII-A High grade, intracompartmental, no metastasesII-B High grade, extracompartmental, no metastasesIII-A Any grade, intracompartmental, with metastasesIII-B Any grade, extracompartmental, with metastasesFigure 25a demonstrates an intramedullary lesion, Figures 25b-c demonstrate a large lesion extending intothe soft tissue, and figure 25e demonstrates lung metastasis. With mets it must be a grade III and theradiographic appearance is typical for osteosarcoma.
    • Question 100A 56 year old man who has a two year history of a progressive peripherial neuropathyhas symmetric motor and sensory deficits in the lower extremities that are worsedistally. Plain radiographs of the spine and pelvis show mulitple small sclerotic lesionsin the pubic rami, left and right ilia, and the lumbosacral spine. Serum electrophoresisshows a monoclonal spike. What is the most likely diagnosis. 1. Metastatic lung cancer 2. Metastatic prostate cancer 3. Osteosclerotic myeloma 4. Non-Hodgkin’s lymphoma 5. Primary hyperparathyroidism
    • Answer: 3This is a classic presentation of osteosclerotic myeloma otherwise know as multiplemyeloma. The major features of this disease are 97% have a homogenous monoclonalspike on serum and urine electrophoresis usually IgG. Sensorimotor polyneuropathyaccompanies leading to severe weakness, atrophy, ataxia. Flat bones of the bodytypically show multiple sclerotic punch out lesions. Malignant plasma cells produceosteoclast activating factor that stimulate osteoclast proliferation and thus rapid bonydestruction.Harrisons 10th Edition Principles of Internal Medicine
    • Question 112Which of the following terms best describe most chondrosarcomas at intitialpresentation? 1. Metastatic 2. Low-grade, intracompartmental 3. Low-grade, extracompartmental 4. High-grade, intracompartmental 5. High-grade, extracompartmental
    • Answer: 2In a study by Memorial Sloan-Kettering Cancer Center most patients presenting withchindrosarcoma complain of pain 76% of the time as their primary symptom. Histologicgrading is divided into Grade I-27%, Grade II-42% (Low grade) and Grade III-31%(high grade). Radiographically the lesion is centrally located 3/3 od the time.
    • Question 123A 36 year-old woman who has had intermittent pain in her knee for the past 8 monthsreports that over the past two months the pain has increased in frequency and intensity.Laboratory studies show that the CBC and ESR are within normal limits. AP and lateralradiographs are shown in Figures 33c and 33d. What is the most likely diagnosis? 1. Lymphoma 2. Osteomyelitis 3. Unicameral bone cyst 4. Aneurismal bone cyst 5. Eosinophilic Granuloma
    • Answer: 2Although CBC and ESR were normal, this can happen with chronic osteo, thehistological slides show osteo and rule out the other choices above. There is a fairlynormal bone matrix (ruling out ABC or UBC) invaded with many small WBC’s in 33c.Slide 33d shows many PMN’s, not lymphocytes, or cosinophils. Making the onlylogical choice to be answer 4. The radiographs offer little help in differentiation, there isperiosteal thickeneing and evidence of an involucrum with lytic lesions although.Ref- Dahlin DC, UnniKK: General aspects and data on 8452 cases, ed 4. Springfield,IL, Charles Thomas, 1986, pp. 448-452.
    • Question 134 -A 14-year-old boy undergoes excisional biopsy of a 3-cm mass over the lateral aspectof the proximal forearm. No imaging studies were obtained prior to the biopsy. Aphotomicrograph of the biopsy specimen is shown in Figure 36. What is the most likelydiagnosis? 1. Desmoid tumor 2. Rhabdomyosarcoma 3. Synovial sarcoma 4. Nodular fasciitis 5. Proliferative fasciitis
    • Answer: 3 In figure 36 a biphasic pattern of spindle cells and epithelial cells should helpto recognize a synovial sarcoma. Synovial sarcoma is most prevalent in adolescentsand young adults between 15-40 years of age. It is more common in males and tends toaffect the extremities (85%-95%).Enzinger F, Weiss S (eds): Soft Tissue Tumors, ed 3. St. Louis, MO, CV Mosby,1995, pp 757-786.
    • Question 139A 56 year old man has had a slowly enlarging soft tissue mass in his left thigh for thepast six months. Plain radiographs show only a soft tissue shadow with nomineralization or obvious bony involvement. The proton density MRI scans shown infigures 39A and 39B show a coronal view and axial view, respectively, of the thigh. Atthis time, management should include 1. excisional biopsy 2. incisional biopsy 3. resection with a wide margin 4. a repeat MRI scan in 3 months 5. a repeat clinical examination in 3 months
    • Answer: 2The MRI study shows a large heterogenous soft tissue mass confined to the lateral compartmentof the thigh without evidence of bone involvement. Biopsy of this suspicious lesion is requiredfor both diagnosis and staging. Soft tissue biopsies can be performed either as a needle biopsy,incisional biopsy, or excisional biopsy. Excisional biopsy is appropriate for small masses whichare believed to be benign. If there is any question of malignancy, incisional biopsy should beperformed through a longitudinal incision placed over the compartment involved in such a waythat the biopsy tract can be removed at the time of the definitive procedure. The clinical historyof a progressively enlarging mass with the MRI findings in figures 39a and b is highly suspiciousand warrants a biopsy. Repeat MRI in 3 months or clinical observation would only delaytreatment. Ref. Enneking WF, Spanier SS, Goodman MA: A system for the surgical staging ofmusculoskeletal Sarcoma. Clin Orthop 1980; 153:106-120.General principles of tumors, in Crenshaw AH, (ed) Campbell’s Operative Orthopaedics, eighthEdition, 1992, pp228-230.Nelson TE, Enneking WF: Staging of bone and soft tissuesarcomas revisited, in Stauffer RN (ed):Advances in Operative Orthopaedics. St. Louis, MO, Mosby Year Book, 1994.
    • Question 140 -A 10 year old boy of Mediterranean ancestry whose height is in the 25th percentilesustains a fracture of the distal femur following a mild fall. Radiographs reveal animpacted fracture of the distal femur as well as osteopenia in both femora and thepelvis. Laboratory studies show a hemoglobin level of 7 mg/dl. A completehematologic evaluation is likely to reveal 1. hemoglobin S and C 2. hemoglobin S chains only 3. no hematologic abnormalities 4. increased total iron binding capacity 5. absence of or severely deficient beta globulin
    • Answer: 5The child described here most likely has thalassemia. This genetically determined hemoglobinopathyseen in patients of Mediterranean descent is characterized by a primary deficiency in the production ofthe beta chains of hemoglobin. The resulting abnormal hemoglobin leads to severe hemolytic anemia.The anemia and secondary hemochromatosis then affect most organ systems including the skeleton.The skeletal changes produced by thalessemia reflect compensatory hyperactivity and hypertrophy ofthe bone marrow, manifested by osteoporosis, widened medullary spaces, and thinned cortices withcoarse reticulations. The clinical skeletal manifestations include arthralgias, delayed skeletalmaturation, premature fusion of the epiphyses in the long bones resulting in short stature, and anincreased incidence of fractures. The fractures heal more slowly than normal, and recurrent fractures atthe same site have been reported. Hemaglobin S chains are indicative of sickle cell disease (HgbS-S) which occurs in 0.3-1.3% ofNorth American Blacks. Sickle cell disease can lead to fractures with minor trauma due to corticalthinning secondary to marrow hyperplasia. The presence of hemoglobin C and S indicates sickle cell- hemoglobin C disease. These patientshave musculoskeletal pain, frequently localized to the joints and have a slightly higher risk ofosteonecrosis of the femoral and humeral head. An increased TIBC suggests iron deficiency anemia. Ref. Dines DM, Canale VC, Arnold WD: Fractures in thalassemia. J Bone Joint urg 1976; 58A:662-666. Resnick D, (ed) Diagnosis of Bone and Joint Disorders, WB Saunders Co, 1995 pp.2107-2137.
    • Question 160 -What is the most common clinical indicator of reflex sympathetic dystrophy of theknee? 1. Effusion 2. Muscle atrophy 3. Atrophic hair changes 4. Disproportionate pain 5. Decreased range of motion
    • Answer: 4Disproportionate pain.O’Brien SJ, Ngeow J, Gibney MA, et al: Reflex sympathetic dystrophy of the knee:Causes, diagnosis, and treatment. American Journal of Sports Medicine 1995;23. pp.655-659.Cooper DE, DeLee JC, Ramamurthy S: Reflex sympathetic dystrophy of the knee:Treatment using continuous epidural anaesthesia. Journal of Bone Joint Surgery1989;71A:365-369.
    • Question 165 -Figures 44a and 44b show the plain radiographs of a 12-year-old boy who has had leftmedial knee pain for the past 4 months. Figure 44c shows representative histologicmaterial. What is the most likely diagnosis? 1. enchondroma 2. osteoblastoma 3. giant cell tumor 4. chondroblastoma 5. osteochondritis dessicans
    • Answer: 4 Chondroblastoma is a lytic lesion commonly located eccentrically in theepiphysis of long bones, most often in skeletally immature individuals. The mostcommon sites of origin are the proximal humerus, proximal and distal femur, andproximal tibia. The radiographic differential includes giant cell tumor crossing into theepiphysis. In this case however, the characteristic highly cellular chondral ovalmononuclear cells seen on the histologic material confirm the diagnosis.Turcotte RE, Kurt AM, Sim FH, et al. Chondroblastoma. Human Pathology 1993;24: 944-949.
    • Question 177A 45-year-old woman who has had increasing foot pain for the past 9 months hastenderness over the region of the cuboid. Oblique and lateral radiographs are shown inFigures 49a and 49b. Low-and high-power photomicrographs are shown in Figures 49cand 49d. What is the most likely diagnosis? 1. Chondroblastoma 2. Giant cell tumor 3. Unicameral bone cyst 4. Aneurysmal bone cyst 5. Metastatic carcinoma
    • Answer: 1Chondroblastoma. Refer above for explanation. Note that there are islands of chondroidmatrix. This is an unusal location for this tumor. Note that chicken wire calcification onthe low power radiograph. This tumor usually occurs in patients less than 20 years old.ABC usually occurs in patients less than 20 years of age. It is eccentric , lytic, expansilearea of bone destruction in the metaphysis. The eseential histo feature is cavernousblood filled without an endothelial lining. UBC most commonly occurs over theproximal humerus. The bone is often expanded. The lesion often appears trabeculated.Tratment can include methyprednisolone injection or curretage and bone graftingBullough et al Atlas of orthopaedic Pathology.
    • Question 186 -A 55 year old man with metastatic prostate cancer has a painful lesion of the midshaftof the humerus in which approx. 75% of the cortex is involved. Management shouldconsist of. 1. an incisional biopsy. 2. a humeral cuff and a sling 3. closed interlocking nailing 4. radiation therapy to the humerus 5. plate fixation with bone fixation
    • Answer: 3Redmond reviewed 13 patients who had 16 pathological fractures of the shaft of thehumerus secondary to metastatic disease. All but one fracture was stabilized withclosed IM nailing. 14 extremities returned to nearly normal function in 3 weeks. Theconclusion stated that IM nailing of the humerus for pathological fractures providesimmediate stability and can be accomplished with a closed technique, brief operativetime, minimum morbidity, with a resultant early return of function to the extremity.
    • Question 190 -A 55-year old woman who has severe pain in her arm for the past 4 months reports thatshe felt a sudden snap in her arm after trying to open a tight jar lid. An AP radiographof the humerus is shown in Figure 53a. A hight power photomictograph of the biopsyspecimen is shown in Figure 53b. What is the most likely diagnosis? 1. Lymphoma 2. Multiple Myeloma 3. Hyperparathyroidism 4. Metatstatic bone disease 5. Mesenchymal chondrosarcoma
    • Answer: 4Dahlin DC, Unni KK: gineral aspects and data on 8452 cases, ed. 4. Springfield, IL,Charles Thomas, 1985, pp 408-413.Reference not found. In this age group (40-80 yrs.), the history alone suggestsmetastatic disease. The X-ray shows an aggressive, permeative, destructive, mixedblastic and lytic lesion with periosteal involvement and soft tissue extension in theproximal diaphyseal humerus associated with fracture. This is uncharacteristic for theother choices. Lymphoma and multiple myeloma are lytic. The high power H&E staindoesn’t have chondroid matrix so mesenchymal chondrosarcoma is unlikely.Hyperparathyroidism typically has osteoclasts and characteristic scalloping seen onmicroscopy. Plus no labs are given to support the diagnosis. Most of this came fromvarious locations in Miller MD, Review of Otrthopaedics 2nd Ed.;WB Saunders, 1996.
    • Question 205Which of the following margins is achieved in a hip disarticulation performed assurgical treatment of a Musculoskeletal Tumor Society (Enneking) type IIA distalfemoral osteogenic sarcoma? 1. Wide 2. Radical 3. Marginal 4. Intralesional 5. Wide-contained
    • Answer: 2Enneking WF: principles of Musculoskeletal Onocologic Surgery, in Evarts CM (ed):Surgery of the Musculoskeletal System, ed 2, New York, NY. Churchill Livingstone,1990, pp 4647-4669Radical margins are aceived when the entire tumor and its compartment (allsurrounding muscles, ligaments and connective tissues) are removed. Marginal line ofresection goes through the reactive zone of tumor, the reactive zone containsinflammatory cells, edema, fibrous tissue, and satellites of tumor cells and have 25 to 50percent local recurrence. Wide margins are accomplished when the entire tumor isremoved with a cuff of normal tissue and have less than 10 percent local recurrence.Intralesional margins go directly through the tumor and result in 100 percent localrecurrence. I dont know what wide contaminated means.A radical margin is definitive for stage II lesions, but is no better than a wide-margin for stage I lesions. If it can be achieved with a logical procedure, radicallocal resection is just as effective as radial disarticulation for stage IIA lesions.Stage IIB lesions in most circumstances require disarticulation to achieve aradical margin.
    • Question 219 -A 35-Year Old man has had increasing pain in the knee for the past 4 months. An APRadiograph of the knee is shown in Figure 58a, and low- and high-powerphotomicrographs of the biopsy specimen are shown in Figures 58b and 58c. What isthe most likely diagnosis? 1. Osteosarcoma 2. Chondroblastoma 3. Giant Cell tumor 4. Aneurysmal bone cyst 5. Desmoplastic fibroma
    • Answer: 350% of Giant cell tumors usually occur about the knee, with 28% involving distalfemur. It is a lytic lesion with periosteal reaction. Histologically giant cells scattereduniformly, and the nuclei of the mononuclear and giant cells are similar in appearance.Most common age is 3rd decade. In this case the radiographic finding are similar toosteosarcoma and Desmoplastic fibroma as well as Giant cell tumor, however,histologic study singles out Giant cell tumor.(Atlas of orthopedic surgery)
    • Question 222 -Figures 60a and 60b show the radiographs of the ankle and distal leg of an 11-year-oldgirl after she twisted her ankle while playing soccer. She has no history of ankle or legpain. Examination reveals localized swelling and tenderness over the lateral ankle, andthe tibia is not tender. The bone lesion identified in the tibia most likely is 1. osteoblastoma 2. osteoid osteoma 3. ossifying fibroma 4. fibrous dysplasia 5. nonossifying fibroma
    • Answer: 5DISCUSSION: Nonossifying fibroma almost always develops in patients under theage of twenty and is usually discovered when a x-ray is taken for an injury.Histologically, there is a triphasic cytological appearance, featuring oval round cells,spindle cells, and giant cells. It usually is a single well-delineated cortical defect withcircular or oval lesions and smooth, lobulated edges. Adjacent body is sclerotic withoutperiosteal reaction. The lower extremity long tubular bones are the most common sitesof occurrence. Treatment is usually not necessary, but occasionally a large lesion willrequire intralesional excision and bone grafting.REFERENCES: Marks KE, Bauer TW: Fibrous Tumors of Bone. Orthop Clin NorthAm 1989; 20(3):377-93.
    • Question 227An 80 year-old man has had increasing hip pain and difficulty ambulating for the past 6months. An oblique radiograph pf the hip is shown in figure 64a, and a technetium bonescan is shown in Figure 64b. Low-and high power photomicrographs are shown inFigure 64c and 64d. What is the most likely diagnosis? 1. Pagets sarcoma 2. Pagets disease 3. Fibrous dysplasia 4. Hyperparathyroidism 5. Metastatic carcinoma
    • Answer: 2Pagets disease is generally discovered in the patient 50 years of age and older. Thisdisease in more common in those of eastern and western European decent and rare inAsians, Scandinavians, and blacks. 20% of patients are asymptomatic and the diagnosisis made while being worked-up for an unrelated complaint. Symptomatic patientscomplain of bone pain, skeletal deformities, changes in skin temperature, pathologicfractures and symptoms related to nerve compression. Radiographic appearance is thatof focal bone resorption and formation in a disordered trabecular pattern, with enlargedcortices and overall bone size. Pagets disease appears hot on technetium bone scans.Treatment is indicated for those with increasing pain and/or deformity. Calcitonin anddiphosphonates are the pharmacologic agents available. Pathologic fractures, disablingarthritis, and severe malalignment are indications for surgical intervention.REFERENCES: Merkow RL, Lane JM: Pagets Disease of Bone. Orthop Clinics NorthAm 1990;21(1):171-89.
    • Question 228 -Which of the following terms best describe most osteosarcomas at the time ofdiagnosis? 1. Metastatic 2. Low-grade, intracompartmental 3. Low-grade, extracompartmental 4. High-grade intracompartmental 5. High-grade extracompartmental
    • Answer: 5DISCUSSION: Osteosarcoma is a difficult disease to cure because it is predominantlyhigh-grade and extracompartmental at the time of presentation, 90% stage IIB. Thecommon presentation is child or young adult with complaints of pain around the knee.The proximal femur and humerus, as well as, the pelvis are not uncommon sites. Pre-operative multiagent chemotherapy is given for 8-12 weeks and then staging studies areperformed. Resection, if possible, is then performed and maintenance chemotherapy isgiven for 6-12 months. Long term survival has increased from 10-20% to 60-70%, withincreased limb salvage.REFERENCES: Enneking WF, Spanier SS, Goodman MA. Clin Orthop 1980;153:106-20.This question deals with the surgical staging of musculoskeletal tumors:STAGE GRADE SITEIA LOW INTRACOMPARTMENTALIB LOW EXTRACOMPARTMENTALIIA HIGH INTRACOMPARTMENTALIIB HIGH EXTRACOMPARTMENTALIII ANY METSUpon diagnosis, most osteosarcomas are high grade, extracompartmental lesions, (stage IIB).
    • Question 231What is the most likely reason that blood for a homologous transfusion that testednegative for the HIV-antibody can carry a low but definite risk of HIV-transmission torecipients? 1. There are many mutations of the HIV virus 2. The test for HIV-antibody is not very accurate 3. The virus may hide in the wall of red blood cells 4. The virus may hide in the wall of white blood cells 5. There is a delay between infection with HIV and the development of a detectable antibody
    • Answer: 5There is a delay between infection with HIV and the development of a detectableantibody. Blood donated in the ―window period‖ may ultimately lead to HIV infectionbecause no antibody formation has occurred thus detection is not possible. HIVmutations may occur but antibodies should develop unless an immunodeficientcondition exists. HIV testing is very sensitive and very accurate with moderntechniques. HIV does not ―hide‖ in the walls of wbc’s and rbc’s. HIV binds to the cellsurface, the viral capsid is released into the cell cytoplasm and ultimately viral DNA isintegrated into the nucleus and host DNA. (AIDS, 3rd Ed. V. DeVita Jr.)
    • Question 232 -What is the most current recommendation for definitive treatment of a 15 year-old boywho has a high grade osteosarcoma of the distal femur? 1. Surgical resection only 2. Raditation therapy only 3. Radiation therapy and surgical resection 4. Chemotherapy only 5. Chemotherapy and surgical resection
    • Answer: 5Chemotherapy and surgical resection combined has lead to a >80% 5 year survival rate.Surgical restriction alone has lead to a high recurrence and metastatic rates and poor(17.4%) survival rates. Radiation therapy alone did not control local recurrences andpulmonary metastasis. radiation and surgery also had high local recurrences andmetastatic rates. Neoadjunctive chemotherapy has provided many improvements intreatment including elimination of micro metastases, necrosis of the primary tumor,reduction of tumor size and neovasculization and helps prevent local recurrences.Complete surgical resection is the mainstay of treatment of osteogenic sarcoma, theaddition of neoadjunctive chemotherapy has proven to be the most effective.
    • Question 243 -The radiograph shown in Figure 67 most likely represents which of the followingdisease processes? 1. Sickle Cell anemia 2. Rheumatoid arthritis 3. Ankylosing spondylitis 4. Degenerative disk disease 5. Diffuse idiopathic skeletal hyperostosis
    • Answer: 3Notice the vertebral squaring and syndesmophytes that are characteristic of AS. Thereis also symmetric blurring of the SI joints. Sickle cell and RA do not have these type ofchanges in the axial skeleton. DDD has disc space narrowing and this x-ray hasmaintained height. DISH can give similar findings, but does not have the SI changesand apophyseal ankylosing.
    • Question 264 -Figure 76 shows the radiographs of a 5-year-old girl who has pain in her left shoulder asa result of a fall from a swing. Management should now include… 1. a biopsy. 2. a CT scan. 3. an MRI scan. 4. a sling and swathe. 5. curretage and bone grafting.
    • Answer: 4 The patient has a pathologic fracture through a unicameral bone cyst of herproximal humerus. As this is a non-weight bearing bone, and a relatively benign lesionyou treat the fracture. Healing of the fracture will occasionally stimulate healing of thecyst. If not then the cyst can be treated with curretage and bone grafting once thefracture has healed.(Green, Swiontkowski, Pathologic Fractures in Children – Skeletal Trauma in Children,1st Edition, 1992.)
    • OITE 1998Question 11Which of the following techniques is most commonly used to classify tissue type insoft-tissue tumors?1. karyotyping2. flow cytometry3. transmission electron microscopy4. immunohistochemistry staining5. scanning electron microscopy
    • Answer: 4Microscopy allows for visualization of the cells; however, special staining is requiredfor identification and classification of specific cells. Karyotyping refers only to thenumber and structure of chromosomes in a cell. Flow cytometry is used to determinethe amount of DNA in a cell, and are predictive for the degree of malignancy. Stainingmethods are currently the most commonly used method to classify tissue type.Enzinger FM, Weiss SW (eds): Soft Tissue Tumors, ed 2. St Louis, MO, CV Mosby,1983, pp 5-9, 83-101.
    • Question 15 -A 55 year-old man has multiple lytic lesions in the humeri, clavicles, and scapulae.Which of the following diagnostic studies best confirms a diagnosis of multiplemyeloma?1. CT scan of the chest2. Bone marrow biopsy3. Complete blood cell count4. Lateral radiograph of the skull5. Erythrocyte sedimentation rate
    • Answer: 2Myeloma is a malignant bone tumor derived from plasma cells. It is associated withabnormalities of protein synthesis. It is the most common primary malignant tumor ofbone. The disease is most common between the ages of 50 and 80. Patients mostcommonly present with bone pain, usually in the spine and ribs. Biopsy is necessary toestablish the diagnosis in a solitary lesion and is still the gold standard for diagnosis ofany lesion. Definitive diagnosis of multiple myeloma is established by bone marrowaspiration. However, multiple myeloma may also be diagnosed with considerableconfidence based on radiographs and lab data. Bence Jones proteins may be found inthe urine. Serum protein abnormalities may cause formation of a rouleaux in theperipheral blood smear. Serum and urine protein electrophoresis are usually abnormal.Answers #3, #4, and #5 may provide some information but the definitive diagnosis isbased on bone marrow aspiration. Answer #1 provides little information.Campbells Operative Orthopaedics. Ninth edition. Volume 1, pp 726-727.
    • Question 17 -Analysis of which of the following proteins is used to establish the diagnosis of Beckermuscular dystrophy?1. Myosin2. Troponin3. Tropomyosin4. Fibrillin5. Dystrophin
    • Answer: 5Becker muscular dystrophy is an X-linked inherited disorder present in approximately 1in 30,000 live male births. The responsible gene is located on the xp21 region of the Xchromosome including sixty-five exons that encode for the protein dystrophin.Duchenne muscular dystrophy is also related to a mutation of the dystrophin gene.Muscle biopsies for dystrophin have been extremely successful for identifying thesedystrophies and distinguishing them from other clinically similar autosomal recessivemyopathies. (Ref: Shapiro, Hoffman)
    • Question 20 -Which of the following radiographic findings would be characteristic of the knee jointsof a patient with neuropathic osteoarthropathy of the knee?1. Fragmentation and subluxation of the normal joint articulation2. Varus deformity with medial subchondral sclerosis3. Preferential narrowing of the medial tibiofemoral compartment4. Narrowing of the medial, lateral, and patellofemoral compartments5. Bone proliferation at the patellar tendon and ligament insertions
    • Answer: 1Neuropathic osteoarthropathy (a.k.a. Charcot joint) develops most often in weight-bearing joints. The most likely cause is diabetes mellitus, but it is also associated withsyphilis, leprosy, yaws, congenital insensitivity to pain, spina bifida,myelomeningocele, syringomyelia, aerodystrophic neuropathy, amyloid neuropathy,peripheral neuropathy of alcoholism, spinal cord injury, peripheral nerve injury, post-transplant neuropathy, and intraarticular steroid injections. The loss of sensation to thejoint is followed by severe degenerative changes, osteophyte formation, articular andsubchondral fractures, and often calcification of surrounding soft tissues. In the knee,this is a tricompartmental disease and will not selectively affect one compartment overanother.
    • Question 45 -A skeletal survey is more accurate than a bone scan for detecting skeletal involvementin which of the following neoplastic diseases?1. Ewing’s sarcoma2. Osteogenic sarcoma3. Multiple myeloma4. Metastatic prostate carcinoma5. Metastatic breast carcinoma
    • Answer: 3Multiple Myeloma lesions are cold on bone scan and because of this a skeletal survey ismore useful.Miller, Review or Orthopaedics; page 292
    • Question 59 -A 60-year-old man with no history of cancer has a destructive lesion in the proximalfemur. He has a long history of tobacco use, but stopped smoking 5 years ago. A needlebiopsy specimen of the lesion shows adenocarcinoma. Which of the following studieswill most likely pinpoint the source of the primary tumor?1. CT scan of the chest2. technetium bone scan3. bone marrow aspiration4. serum protein electrophoresis5. lateral skull radiograph
    • Answer: 1Skeletal metastasis of unknown origin - ...plain radiographs of the chest established thediagnosis of carcinoma of the lung in seventeen patients (43%).......Unlike skeletal metastasis of known origin - most often breast or prostate - a metastasisof unknown origin usually originates in the lung or kidney. In the present series, themost common occult primary site was the lung (63%) and the second most commonwas the kidney (10%).Rougraff BT, Kneisl JS, Simon MA: Skeletal metastasis of unknown origin: Aprospective study of a diagnostic strategy. LBLS 1993; 75-A:1276-1281.
    • Question 60 -What factor is most commonly associated with malignant transformation of a giant celltumor?1. high-grade histology of the initial tumor2. multiple local recurrences after curettage3. previous treatment of the tumor with cryotherapy4. previous treatment of the tumor with radiation therapy5. extraosseous extension into two or more adjacent compartments
    • Answer: 4Most authors have agreed that radiation therapy should be avoided in the treatment ofgiant cell tumor, as there is a high prevalence of sarcomatous degeneration.Gitelis S, Wilkins R, Conrad EU II: Benign bone tumors, in Pritchard DJ (ed):Instructional Course Lectures 45. Rosemont, IL, American Academy of OrthopaedicSurgeons, 1996, pp425-446. (p440)
    • Question 69 -Crush fractures of the vertebral body are a particularly common problem in type 1(postmenopausal) osteoporosis because1. Trabecular bone is preferentially resorbed in this high bone turnover state2. Loss of water content in the disk increases impact load to the vetrebral bodies3. Stress is imposed by the relative stiffness of the arthrtic facet joints4. Increased energy demands are imposed by decreased circulation to the vertebral body5. The thick cortical bone found in the vertebral body resorbs rapidly following estrogen withdrawal
    • Answer: 1 The spine is composed primarily of trabecular bone, compared with corticalbone, it has a high surface-to-volume ratio. Because metabolic activity (remodeling)occurs on bone surfaces, trabecular bones in general and the vertebral bodies inparticular are resorbed preferentially in times of skeletal loss. Osteoporosis ischaracterized by trabeculae of decreased size and number. It has also beendemonstrated in osteoporosis that there is a thinning of the cortex as well as a change inthe shape of the trabecular bone from plates to narrow bars. In the phases of bone loss,vertebral body density declines before a similar loss is detected in cortical areas. Thebody accommodates bone loss by redistribution. A 10 percent shift of bone massoutward from the epicenter through an enlargement of the bone diameter willcompensate for 30 percent decrease in the bone mass against applied bending andtorque stresses but not against axial loading. This differential resorption explains thetiming and patterns of the fracture syndromes seen in osteoporosis. The incidence ofvertebral crush fractures rises immediately after menopause, secondary to hightrabecular content and estrogen deficiency. (precise mechanism of effects of estrogenwithdrawal unclear)Bernstein J, Lane JM: Metabolic bone disorders of the spine, in Rothman RH, SimeoneFA (eds): The Spine, ed 3. Philadelphia, PA, WB Saunders, 1992, pp. 1381-1427.
    • Question 77 -Which of the following types of sarcoma of the bone is most sensitive to external beamradiation?1. Ewings tumor2. Parosteal osteosarcoma3. Dedifferentiated chondrosarcoma4. Low grade intramedullary chondrosarcoma5. High grade intramedullary osteosarcoma
    • Answer: 1Parosteal osteosarcoma occurs on the surface of the metaphyseal regions of the distalfemur or the proximal humerus most commonly. The treatment is wide surgicalresection versus limb salvage. Dedifferentiated chondrosarcoma has a moth eatenappearance and may occur as a transformation of chondrosarcoma. Treatment isresection and prognosis is poor. Low-grade intramedullary chondrosarcoma is alsotreated with surgical resection. High-grade intramedullary osteosarcoma is usuallytreated with pre-op chemo and resection. The only tumor listed where radiation is anoption is Ewings tumor.
    • Question 102 -Flow cytometry of tumors measures the1. Size of cells2. Amount of DNA in cells3. Nucleus-cytoplasm ratio4. Specific DNA sequences5. Specific messenger RNA sequences
    • Answer: 2Flow cytometry is a method of quantitating components or structural features of cellsprimarily by optical means. Ploidy and cell cycle analysis of cancers is the majordiagnostic use. Cells are passed single file through a laser beam by continuous flow andseveral parameters are measured including Cell Diameter, proportional quantity ofgranular (DNA) within the cell, and using fluorescent probes the total DNA or aspecific DNA/mRNA sequence can be counted. In examining tumors the amount ofDNA in each cell is important for determining neoplasia. Ref. www.bio.umass.edu/mcfacs/intro.htm, the U. of Massachusetts web site on flowcytometry.
    • Question 111 -A 47-year-old woman who reports mild, aching pain in her knee has no history oftrauma. Examination of the knee is normal. Figure 23a shows the AP radiograph. Abone scan shows increased uptake at this site only. Figure 23b shows the CT scan, andFigure 23c shows the histology from the CT scan-guided needle biopsy. Treatmentshould include1. extended curettage and polymethylmethacrylate cementation2. extra-articular resection of the knee and an allograft arthrodesis3. wide resection of the proximal tibia and custom prosthetic replacement4. prophylactic internal fixation and postoperative irradiation5. excision of the lateral condyle and reconstruction with a hemicondylar allograft
    • Answer: 1Patients with giant cell tumors typically present with local pain, swelling andtenderness. Radiographs usually reveal a lesion destructive of both medullary andcortical bone. The lesion is characterized by an expanding zone of radiolucency that islocated eccentrically in the end of a long bone in an adult.The modern technique for the removal of a giant cell tumor involves wide decorticationof all the bone overlying the area of the tumor. The cavity is filled withmethylmethacrylate bone cement and covered with demineralized bone matrix tostimulate the restoration of strong cortical boundaries. The other procedures are muchmore invasive and not necessary to treat a low-grade neo-plastic lesion such as a giantcell tumor. Fewer complications and better functional results have been found afterintralesional excision and insertion of methylmethacrylate than other techniques.Gitelis S, Wilkins R, Conard EU II: Benign bone tumors. In Pritchard DJ: InstructionalCourse Lectures 45,1996.
    • Question 123Which of the following types of osteosarcoma is associated with the best prognosis forlong-term survival?1. Parosteal2. Periosteal3. High-grade intramedullary4. Osteosarcoma occurring in Paget’s disease5. Osteosarcoma occurring in irradiated bone
    • Answer: 1 p. 194 (parosteal): Early adequate treatment [of parosteal osteosarcoma]should lead to cure in most patients. A long-term survival rate of 80%-90% is to beexpected for patients who have parosteal osteosarcomas without dedifferentiation.‖ p. 163 (in Paget’s disease): ―Although long-term survival is rare for patientswith this type of sarcoma, four patients have survived more than 10 years.‖ p. 164 (in irradiated bone): ―The location of these tumors in unresectablelocations such as the skull, clavicle, scapula, and spine explains the traditionally poorprognosis.‖Unni, Dahlin’s Bone Tumors, 5th ed., 1996.
    • Question 135 -Which of the following metastatic tumors to bone carries the greatest risk ofcomplications from intraoperative bleeding?1. Breast2. Prostate3. Gastrointestinal4. Kidney5. Multiple myeloma
    • Answer: 4Just remember that renal cell CA is extremely vascular, and that an preopembolization should be performed prior to the procedure in order to minimizethe blood loss.
    • Question 142 -A form of renal osteodystrophy that is characterized by pure osteomalacia is caused by1. Secondary hyperparathyroidism2. Phosphate retention secondary to uremia3. Insufficient renal synthesis of 1, 25 dihydroxy vitamin D4. Aluminum deposition in bone from oral phosphate binders5. Persistent acidosis aggravating the negative calcium balance
    • Answer: 4There are many causes of rickets and osteomalacia. Renal osteodystrophy is a commoncomplication of chronic renal failure and is one of the most common causes ofosteomalacia. Pure osteomalacia is caused by the aluminum in phosphate binders usedto treat hyperphosphatemia in renal failure. Desferoxamine is an effective chelator ofaluminum in patients with biopsy documented aluminum-associated osteomalacia. Pureosteomalacia also can be caused by hypophosphatemia. The other choices are part ofthe mechanism of bone changes in renal osteodystrophy.(Simon: Orthopedic Basic Science, 1994 pp168-172)
    • Question 146Figure 30 shows the current radiograph of a 32-year-old woman who had a giant celltumor of the distal radius that was treated with curettage/burring and packed withpolymethylmethacrylate 2 years ago. What is the most likely diagnosis?1. Osteomyelitis2. Malignant degeneration3. Stress fracture4. Local recurrence of the giant cell tumor5. Bone resorption due to methylmethacrylate
    • Answer: 4Number four is the correct answer because recurrence is the most likely cause of thelytic zone. Since cement resists invasion by the tumor, lysis of the surrounding bone isinevitably produced in a recurrence. A 1-2 mm lytic zone can be found normally,greater than 5 mm lytic zone is positive for recurrence and the 3 – 5 mm lytic zone isvery suspicious and should be followed by MRI and image guided needle biopsy. Noother sign of osteomyelitis or stress fractures are noted.
    • Question 153Lymphatic metastasis is a common feature of which of the following lesions?1. Liposarcoma2. Nodular fasciitis3. Rabdomyosarcoma4. Malignant fibrous histiocytoma5. Extra-abdominal desmoid tumor
    • Answer: 3Rhabdomyosarcoma is a high grade malignancy with a rapid growth pattern. Localrecurrence and distant metastasis after inadequate excision occurs in almost allinstances and is uniformly fatal. The primary site of metastasis is the lung. Lymph nodemetastasis occurs in about > 10% of the patients, (synovial cell sarcoma = 25%).Whereas M.F.H. only occasionally metastasizes to regional lymph nodes, andliposarcoma is slow growing and recurrences or local metastasis is < 10%.Reference: Enneking, W.F. et al. Clinical Musculoskeletal Pathology , University ofFlorida, pp. 16-1-16-46.
    • Question 170Which of the following surgical options after resection of a sarcoma about the kneewould require a patient to expend the greatest amount of energy while walking?1. Arthrodesis2. Rotationplasty3. Above-knee amputation4. Osteoarticular allograft5. Endoprosthesis (custom arthroplasty)
    • Answer: 3The answer to this question is based on a study by Harris which tested the effeciency ,rate, and percent of max rate in ambulation, in amputees, those with arthrodeses andarthroplasties. They found that Above knee amputees expended the most energy,followed by arthrodesis patients, and then arthroplasty patients.Reference: Harris IE, Leff AR, Gitelis S. et al: Function after amputation, arthrodesis,arthroplasty for tumors about the knee. JBJS 1990; 72A:1477-1485.
    • Question 186 -Which of the following metastatic carcinomas has the worst long-term prognosis?1. Lung2. Breast3. Prostate4. Thyroid5. Renal
    • Answer: 1The answer is the lung, which has a dismal long-term prognosis. However, not included in thequestion, but reviewed in the cited reference and worthy of noting is Gastric CA. This has theworst prognosis with a 15% survival rate of 6 months after spinal metastasis is detected.Recommendations are palliative care rather than surgical intervention.Pulmonary has 6-month & 1-year survival rates of 15% & 22% respectively. Surgicalintervention will be based on the overall condition of the patient and when indicated the extent ofsurgical intervention should be as small as possible.Breast & prostate CA have better survival rates than the others, and are often hormonallysensitive tumors. Making endocrine therapy initially crucial with the good long term survival ofprostate CA patients. When intervention is indicate, it should be combined with other modalities(Chemo, Etc.) and a more extensive surgery including decompression and fixation, but alsoresection of the metastatic lesion and reconstruction.Breast CA rarely progresses with symptoms due to spinal metastasis, likely due to symptomsbeing controlled with hormone and radiation therapy.Renal CA has a poor 6 months survival rate of 51% which is close to lung CA, but a sharpdecline in deaths from 6-36 mos results in a high (40%) 3-year survival rate. These should becategorized into rapid growing & slow growing with surgical intervention based on thisclassification.Reference: Tatsui H, et al. Survival rates of patients with metastatic spinal cancer afterscintigraphic detection of abnormal radioactive accumulation. Spine 1996;21:2143-48.
    • Question 187 -The Magnetic resonance imaging signal characteristics of a high-grade soft-tissuesarcoma are best described as1. T1-low, T2-low.2. T1-low, T2-high.3. T1-moderate, T2-low.4. T1-high, T2-low.5. T1-high, T2-high.
    • Answer: 2After plain radiographs of the affected area have been obtained, magnetic resonance imagingmodality is the best imaging modality for detecting and characterizing the lesion, regardingdefinition of normal muscle, fascial boundaries, and the tumor mass. Although MR imaging isnot specific in determining whether lesions are benign or malignant, it can be useful inevaluation other characteristics, such as size, pattern of growth, integrity of natural boundaries,and homogeneity.Intravenous contrast agents are not necessary to evaluate neurovascular structures. Both the T1& T2 weighted images are essential to detect and characterize soft tissue lesions.Most Tumors have long T1 and T2 relaxation times, therefore, in most instances signal intensityalone is of limited benefit. Exceptions are lipoma, hematoma, intra-lesional hemorrhage.Hemorrhage may occur in some soft tissue lesions, especially sarcomas, leading to the low T1and high T2 intensity sound on MR. Miller, indicates that Water, CSF, acute hemorrhage and softtissue tumors appear dark on T1 and light on T2.Reference: Soft-Tissue Tumors: Diagnosis, Evaluation, and Management. JAAOS 1994;2:202-211.Sundaram, M, The Role of MRI in evaluation of Muskuloskeletal Tumors, Radiology: Diagnosis-Imaging_Intervention Vol 5: 104; 6-10.
    • Question 210 -The radiograph shown in Figure 50a and the CT scan shown in Figure 50b reveal alesion in the left femoral neck of a 12-year-old boy who has pain in the left hip. Themost likely cause of the osteopenia of the left proximal femur is1. disuse osteopenia2. paraendocrine effect of the tumor3. abnormally increased density on the right side4. side effect of the treatment of the lesion5. extensive tumor involvement of the left hip
    • Answer: 1Figure 50A: AP pelvis with a 1 cm lucency in the calcar region of the femoral neck anddiffuse osteopenia of the proximal femur. Figure 50B: CT scan of the same patientshowing a well demarcated lcm lesion in the femoral neck with an obvious nidus.Careful evaluation of the radiographs is critical in this question. First the obviousosteoid osteoma must be recognized. From there answers 2 and 3 can be eliminated.Neither radiograph reveals any sign that treatment has occurred, and the CT scan showsno signs of extensive involvement, thus answer 5 should also be eliminated. The realkey is believing that such diffuse osteoporosis could occur in such a young child, asJones described in his article from 1969.Jones G: Radiological appearances of disuse osteoporosis. Clin Radiol1969;20:345-353.
    • Question 224 -The diagnostic distinction between a benign enchondroma and a low-gradeintramedually chondrosarcoma is based primarily on the1. clinical history and radiographic findings.2. technetium bone scan3. flow cytometry pattern of extracted chondrocytes4. immunohistochemical staining patterns of a biopsy specimen5. histologic features of a biopsy specimen stained with hematoxylin-cosin
    • Answer: 1"The diagnostic strategies for benign bone tumors center on the initial radiographicpresentation." … "For example calcified, lytic phylangeal lesionstrongly suggests the presence of cartilage that is consistent with enchondroma."
    • Question 246 -Figures 66a and 66b show the radiographs of an 8-year-old girl who has a firm,immobile mass of her middle finger at the proximal interphalangeal joint. Figure 66cshows the histopathology of the biopsy specimen. What is the most likely diagnosis?1. Fracture healing2. Chondrosarcoma3. Periosteal chondroma4. Periosteal osteosarcoma5. Dysplasia epiphysealis hemimelica
    • Answer: 3 Periosteal chondroma is a benign chondroid tumor that arises under or in theperiosteum on the surface of cortical bone. Has a scalloping of the cortex with a welldefined margin between the tumor and bone. The tumor bed shows a variable amount ofsclerosis. Also a variable amount of calcification exists. Histologically, as in this case,there is chondroid matrix. The predilection for the proximal end of long bones,particularly the humerus, and the bones of the hands, has been noted. This tumor mustbe differentiated from periosteal chondrosarcoma, which penetrates the cortical boneand is generally larger with a soft tissue mass. Histology will also show morepolymorphism and hyperchromatic nuclei of chondrosarcoma.Boriani. JBJS 1983;65A:205-212.
    • Question 262A 45-year-old woman has a deep soft-tissue mass in the thigh. The MRI scan Shows a12-cm heterogenous mass that is deep to the fascia, and the CT scan Shows three small(5 to 10 mm) peripheral, noncalcified pulmonary nodules. What is the stage of diseaseaccording to the staging system of the Musculoskeletal Tumor Society?1. 12. 23. I4. II5. III
    • Answer: 5The system adopted by the Musculoskeletal Tumor Society is based on three factors:Grade (G), Site (T), and metastases (M). Each of these factors is stratified bycomponents that influence both prognosis and response to treatment. Grade is anassessment of biologic aggressiveness of the legion. There are three stratifications ofGrade: G0 = benign, G1 = low grade malignant (few mitoses, moderate differentiation,and distinct matrix), G2 = high grade malignant (frequent mitoses, poorly differentiatedcells, and sparse immature matrix). Site is the anatomic setting of the legion,determinedby radiographic imaging. There are three strata T0 = intracapsular (does not extendbeyond the compartmental boarders of its origin and remains completely within itscapsule) T1 = extracapsular, intracompartmental, (extends beyond its capsule butremains within its compartment), T2 = extracapsular, extracompartmental, (extendsbeyond its compartmental boundaries). Metastasis has two strata M0 = no evidence ofregional or distant mets and M1 = regional or distant mets. Stages for benign tumorsare listed as 1,2 and 3 whereas stages for malignant tumors are I, II, and III.
    • StageGrade Site MetsBenign tumors Stage 1, latent G0 T0 M0 Stage 2, active G0 T0 M0 Stage 3, aggressive G0 T0 M0Malignant Tumors Stage I, low grade A. Intracompartmental G1 T1 M0 B. Extracompartmental G1 T2 M0 Stage II, high grade A. intracompartmental G2 T1 M0 B. extacompartmental G2 T2 M0Stage III, metastaticA. intracompartmentaldistant mets G1-2 T1 M1B. extracompartmental distant metsG1-2 T2 M1By the nature of the radiographic appearance of this person’s tumor andassuming we are dealing with a malignant tumor with metastases, this has to be a stage III tumor.
    • Question 269 -A patient who has a malignant bone tumor of the hip undergoes resection and hiparthrodesis. While walking, mean oxygen consumption would be approximately whatpercent of that observed in someone who has a normal hip?1. 50%2. 100%3. 130%4. 200%5. 300%
    • Answer: 3―Unilateral arthrodesis has been shown to increase O2 consumption in normal gait by32%.‖
    • OITE 1999Question 8Figure 2 shows the plain AP radiograph of a 20-year-old man who has had apainful swelling in his left wrist for the past 4 months. A bone scan shows asolitary lesion. A radiograph and CT scan of the chest are normal. Prior tobiopsy, what is the most likely diagnosis?1. Telangiectatic osteosarcoma2. Chondromyxoid fibroma3. aneurysmal bone cyst4. giant cell tumor5. chondroblastoma
    • Answer: 4Bones involved are the distal femur, proximal tibia, distal radius. other longbones. On x-ray a lytic lesion in the epiphysis without surrounding sclerosis orperiosteal bone reaction. Often has a "soap bubble" pattern. Treatment is enbloc excision, if possible, but usual treatment is currettage.
    • Question 17 -Which of the following is considered the most common primary carcinoma tometastasize to bones distal to the elbow and knee?1. Breast2. Prostate3. Kidney4. Lung5. Thyroid
    • Answer: 4Axial skeletal involvement is more common than appendicular involvement, andmetastatic lesions below the elbow and knee are relatively uncommon.Autopsy studies on patients with metastatic disease below the knee and elbowrevealed that lung was the most common primary site. The tibia was the mostcommon bone affected. Pain, local soft-tissue extension and pathologicfractures were the most common presenting symptoms.Reference: Leeson MC, Makley JT, Carter JR: Metastatic skeletal diseasedistal to the elbow and knee. Clin Orthop 1986;206:94-99.
    • Question 25Which of the following signal sequences describes the MRI scan characteristicsof normal tendons?1. Low on T1-weighted images and low on T2-weighted images2. Low on T1-weighted images and high on T2-weighted images3. High on T1-weighted images and low on T2-weighted images4. High on T1-weighted images and moderate on T2-weighted images5. High on T1-weighted images and high on T2-weighted images
    • Answer: 1 T1 sequenced images are weighted toward fat and T2 sequencedimages are weighted toward water. Consequently, normal tendons, ligaments,and cortical bone all appear low signal on all pulse sequences.Beaty JH (ed): OKU 6. Rosemant IL, AAOS, 1999, 9981-87.
    • Question 36 -Which of the following conditions will most likely eventually develop in anewborn girl with achondroplasia?1. Degenerative disease of the hips2. Spinal stenosis3. Atlantoaxial instability4. Cardiomyopathy5. Patellar subluxation
    • Answer: 2Achondroplasia is the most common type of short-limb disproportionatedwarfism. The primary defect found in achondroplastic dwarfs is abnormalenchondral bone formation. Periosteal and intramembranous ossification isnormal. Spinal deformities are the most common and potentially disablingproblem for the achondroplastic dwarf. The spinal canal is developmentallynarrowed, particularly in the lower lumbar segments. Stenosis of the spinalcanal and intervertebral foramen is secondary to short, thickened pedicles,interpedicular narrowing, thickened laminae, and inferior facets. Additionalfactors, such as intervertebral disc herniation, degenerative spondylolysis,excessive lumbar lordosis, or anterior wedging of the vertebral bodies from athoracolumbar kyphosis, narrow the canal further.Reference: Bassett GS: The osteochondrodysplasias, in Morrissey RT,Weinstein SL 9eds): Lovell and Winter’s Pediatric Orthopaedics, ed 4.Lippincott-Raven, 1996, pp 203-223.
    • Question 43 -A 75-year-old man has a destructive lesion of the proximal femur with soft-tissue extension. A technetium bone scan shows no other lesions, and a CTscan of the chest is negative. A needle biopsy of the soft-tissue componentshows high-grade chondrosarcoma. According to the staging system of theMusculoskeletal Tumor Society, the stage of the lesion is1. IB2. IIB3. III4. 25. 3
    • Answer: 2Grade is an assessment of the biological aggressiveness of the lesion, which isa blend of histology, radiographic assessment, and clinical reflection.G0=benign, G1=low grade malignant, G2=high grade malignantChondrosarcoma is considered high grade malignant. The anatomic setting orSite (T) has a direct relationship to the prognosis and the choice of surgicalprocedure. These are determined primarily by clinical and radiographictechniques. T1=intracapsular, T2= extracapsular, intracompartmental,T3=extracapsular, extracompartmental. In most staging systems forcarcinomas metastatic involvement is stratified as either regional (N) or distant(M). For sarcomas both have the same ominous prognosis and both aredesignated (M). M0=no regional or distant mets, M1=regional or distant metspresent. The three factors of grade, site, and metastasis are combined to formthe criteria for the progressive stages of benign and malignant lesion. Benignlesions are designated by the Arabic numerals 1,2,and 3 corresponding tolatent, active, or aggressive lesions. Stages of malignant lesions aredesignated by the Roman numerals I,II, or III, and these stages aresynonymous with low-grade, high-grade, and metastatic. The three states ofsarcomas are further stratified into A or B depending on whether the lesion isanatomically intracompartmental (A) or extracompartmental (B).
    • Question 48Figure 8 shows the AP radiograph of the sacroiliac joints of a 29-year-old manwho has had increasing lower back pain and stiffness for the past 4 months.Management consisting of exercise and nonsteroidal anti-inflammatory drugshas provided only minimal relief. Examination reveals decreased lumbarmobility in all directions, normal sensibility, and no weakness. The most usefulphysical finding for confirming the diagnosis is1. a positive straight leg raising test.2. limitation of chest expansion to 1" or less.3. the presence of urethritis.4. bilateral tightness of the hamstrings.5. unilateral absence of the Achilles reflex.
    • Answer: 2The AP radiograph of the patients sacroiliac joints shows sclerosis in thebilateral SI joints indicative of sacroiliitis. This is a hallmark finding in ankylosingspondylitis as are the rest of the symptoms described in the question. Patientswith AS often have other joint involvement such as pain from inflammation ofthe costovertebral joints which inhibits chest expansion. Eventual ankylosis ofthese joints may result in severe fixed limitations of chest expansion. A positivestraight leg-raising test is a tension sign that is suggestive of a herniated disc,yet the patient has normal sensibility and no weakness in his lower extremities.This would also exclude unilateral absence of the Achilles reflex. The presenceof urethritis would be suggestive of Reiters. Bilateral tightness of thehamstrings may be a finding but is not a useful finding for confirming thediagnosis.Reference: Frymoyer JW (ed): The Adult Spine: Principles and Practice.Philadelphia, PA, WB Saunders, 1992, pp 699-705.El-Khoury GY, Kathol MH, Brandser EA: Seronegative spondyloarthropathies.Radiol Clin North Am 1996;34:343-357.
    • Question 63 -Figure 13 shows the AP radiograph of both knees of a 55-year-old man whoreports an acute exacerbation of right knee pain for the past 36 hours.Examination reveals a palpable effusion, moderate warmth, and pain on rangeof motion. A synovial fluid aspiration will most likely show1. A WBC greater than 100,000/mm32. Gram-negative intracellular diplococcus3. Positive birefringent crystals4. Negative birefringent crystals5. Elevated complement levels
    • Answer: 3 Figure 13 shows chondrocalcinosis of bilateral knee menisci, whichmay be caused by several disorders, including calcium pyrophosphatedeposition (CPPD), ochoronosis, hyperparathyroidisim, hypothyroidisim, andhemechromatosis. CPPD (a.k.a. pseudogout) is a common disorder ofpyrophosphate metabolism that occurs in older patients and occasionallycauses acute attacks, usually in the lower extremities, especially the knee.Short, blunt (rhomboid-shaped) rods that are weakly positively birefringent aredemonstrated following aspiration. Negative birefringent crystals are seen ingouty knee aspirates. Answers 1 and 2 are seen in septic knee aspirates,which with this patient’s symptoms, should be in the differential diagnosis.However, the radiograph is more suggestive of pseudogout condition.
    • Question 74Which of the following benign lesions may occasionally metastasize to thelungs.1. Nonossifying fibroma2. Osteofibrous dysplasia3. Chondromyxoid fibroma4. Chondroblastoma5. Periosteal chondroma
    • Answer: 4Chondroblastoma is a rare benign neoplasm of fetal-type cartilagedifferentiation. Over 50% of the lesions occur in patients between the ages of15 and 25, with 70% occurring in the long bones. Radiologically,Chondroblastoma is usually a well-circumscribed lytic lesion that, when in along bone, involves the epiphysis. Histologically, Chondroblastoma iscomposed of sheets of stromal cells, scattered multinucleated giant cells, andvarying amounts of chondroid matrix. The stromal cells, resembling fetalchondroblasts, are distinctive. This benign, slow growing neoplasm is besttreated with curettage and bone grafting. Rarely, these are locallydestructive. Another rare behavior is pulmonary metastasis, the so-called―benign metastasizing Chondroblastoma.‖ Growth of these rare metastatic fociis very slow, and they are usually treated successfully by pulmonary wedgeresections.McCarthy EF, Frassixa FJ (eds): Pathology of Bone and Joint Disorders withClinical and Radiological Correlation. Philadelphia, PA, WB Saunders, 1998,p221.
    • Question 95In which of the following pediatric neoplasms can involvement of the synoviumresult in arthralgias?1. Ewing’ tumor2. Osteosarcoma3. Eosinophilic granuloma4. Leukemia5. Chondrosarcoma
    • Answer: 4Bone and joint symptoms are common presenting complaints in children withacute leukemia. These include bone tenderness, swelling, and arthralgias.Arthralgias, which symmetrically involve multiple joints, are caused by leukemicinfiltration of the synovial membrane. Synovial involvement is minimal tononexistent in the other forms of cancer above.(Pathology of Bone and Joint Disorders with Clinical and RadiologicCorrelation, 1998, 127-128; Annals of Internal Medicine, 1963)
    • Question 104Which of the following devices provides the best prophylactic stabilization of animpending pathologic fx caused by a lytic defect in the mid-diaphysis of thefemur?1. plate and screws alone2. plate and screws with cement3. Rush rod with cement4. Ender’s rods with cement5. locked intramedullary nail
    • Answer: 5Impending pathologic fx of the femoral shaft can usually be treated byconventional closed IM rodding techniques. The use of reconstruction nails,locked prox. And distally, has served to reduce the common complication ofprogressive fem collapse with telescoping of the fx fragments and proxmigration of the rod. Prox placement of screws into the fem head offers moresecure fixation than conventional transverse or antegrade screw placement andmay protect the femoral head from subsequent fx. Both impending lesions and complete pathologic fx of femoral head andneck should be managed with replacement arthroplasty. Pathologic fx insupracondylar and condylar regions of femur are unusual and difficult to tx. Ifthere is sufficient bone stock, use of conventional internal fixation devicesaugmented with methylmethacrylate will usually achieve stability. Tx principlesare the same regardless of location. A construct should ideally provide enoughstability to allow immediate full weight bearing withenough durability to last the patient’s expected lifetime.
    • Question 112 -Figures 25a and 25b show the AP and lateral radiographs, and Figures 25c and25d show the T1 and T2 weighted MRI scans of a 42 year old man who has apainful mass of the ankle. Figure 25e shows a histopathologic specimen. Whatis the most likely diagnosis?1. Aneurysmal bone cyst2. Giant cell tumor3. Hemangioma4. Osteomyelitis5. Intraosseuos ganglion
    • Answer: 5Inraosseous ganglions are also known as subchodral bone cysts. They typically occur inthe 2nd – 6th decades of life and are more common in males. On plain radiographsinraosseous ganglia appear as well defined, oval or round osteolytic areas with a thinrim of sclerotic bone. On MRI, there is a low signal on T1 and high on T2. Histologicalfeatures include stellate or fibroblast-like cells that produce a large amount of mucoidground substance.Aneurysmal bone cysts are eccentric, lytic, expansile areas of bone destruction on x-ray.The essential histologic features are cavernous blood filled spaces without anendothelial lining. This is not seen in Fig. 25e.Giant cell tumors reveal a purely lytic destructive lesion in the metaphysis. The basicproliferating cell has a round or oval shaped nucleus. Numerous mitotic figures are alsoseen. Hemangiomas are soft tissue tumors that can occur in a cutaneous,subcutaneous, or intramuscular location. Plain radiographs may reveal small phleboliths.On MRI, heterogeneous lesions with numerous small blood vessels are seen.There multiple forms of osteomyelitis and most are easily recognized clinically,radiographically and histologically. Most are located in the metaphyseal region and showvarying degrees of a lytic process with some degree of sclerosis.Helwig U, Lang, Baczynski,: The intraosseous ganglion: A clinical pathologic report on42 cases. Arch Orthop Trauma Surg 1994;114: 14-17.M. Forest, Tomeno, Vanel, Orthopedic Surgical Pathology, 1998, pp547-552.
    • Question 118 -Which of the following soft-tissue tumors has the following MRI scan signalcharacteristics: high signal on T1-weighted images and intermediate to highsignal on T2-weighted images?1. Synovial sarcoma2. Extra-abdominal desmoid tumor3. Malignant fibrous histiocytoma4. Lipoma5. High-grade pleomorphic liposarcoma
    • Answer: 4 Magnetic resonance imaging has become the most useful modality forthe definition of soft tissue masses. Although the MR image can detect softtissue masses with a very high sensitivity, it is not possible to accurately predictthe histology or whether a lesion is benign or malignant. The two exceptions tothis general rule are lipomas and hemangiomas. Lipomas often are veryhomogeneous and have signal characteristics that exactly match those of thesurrounding fat, high signal on T1-weighted images and intermediate to high onT2-weighted images, thus establishing the diagnosis.Reference: Frassica FJ, Frassica DA: Soft tissue tumors: Diagnosis,evaluation, and management. J AM Acad Orthop Surg 1994;2:202-211
    • Question 132Figures 31a and 31b show the radiograph and biopsy specimen of a 57-year-old man with no history of trauma who has had painful swelling of the distalphalanx of the little finger for the past 2 months. What is the most likelydiagnosis?1. Osteomyelitis2. Epidermal inclusion cyst3. Metastatic lung carcinoma4. Metastatic kidney carcinoma5. Metastatic prostate carcinoma
    • Answer: 3 ―Acrometastases‖ is a term first used by German authors about 25years ago to describe metastatic lesions occurring in the hands and feet. Thefiltering mechanism of the lungs may partially explain why the lung tumor cellsbypass the lung-filtering activity by directly invading pulmonary vessels,reaching the left heart and then spreading to remote regions. Therefore, lungtumors theoretically are more likely to be associated with acrometastatictumors. Carcinomas of any origin can spread to bone, the most common arecarcinomas of lung, breast, prostate, kidney, or thyroid. Carcinomas of theprostate, breast, and kidney secondary to ―batson’s plexus‖ is a major factor inthe development of spinal metastasis. Pelvic bone metastasis of prostatecancer is common secondary to proximity. The figures do not show thecharacteristics of osteomyelitis of cortical destruction and formation ofperiosteal new bone. Epidermal inclusion cysts do not pertain to figures shown.Reference: Lombardi RM, Amadio PC: Acrometastases, in Sim FH (ed):Diagnosis and Management of Metastatic Bone Disease: A multidisciplinaryApproach. New York, NY, Raven Press, 1988, pp. 237-243.
    • Question 145 -A 73-vear-old woman has had aching pain in the right ankle region for the past4 months. Results of serum and urine protein electrophoresis show normalmigration. Figures 34a through 34c show the plain lateral radiograph of thetalus, sagittal TI-weighted MRI scan, and biopsy specimen. What is the mostlikely diagnosis?1. Metastatic carcinoma2. Lymphoma3. Multiple Myeloma4. Fibrosarcoma5. Malignant fibrous histiocytoma
    • Answer: 2The referenced films show an x-ray with a lytic lesion and periosteal elevationof the talus, an MRI with decreased signal on T1, and a photomicrograph withabundant mononuclear cells in various stages of division. Given the negativeSPEP/UPEP, the only possible correct response is Lymphoma. Bone tumorscaused by Lymphoma have the above characteristics and are mostly found inthe Femur, Pelvis, and Skull. Involvement of the talus is rare.Reference: Huvos, Bone Tumors 2d Edition, 625-637
    • Question 157 -Figures 38a through 38c show the plain radiograph and coronal T1- and T2 -weighted MRI scans of a 30-year-old man who has a painful lump over thedistal deltoid on the lateral aspect of his arm. The mass is marginally excised,and a histologic section at the periphery of the mass is shown in Figure 38d.Management should include1. observation.2. serial follow-up staging studies.3. radiation therapy.4. wide reexcision, followed by radiation therapy.5. chemotherapy.
    • Answer: 1Osseous soft tissue tumors are either extraskeletal osteosarcomas or myositisossificans. If they specifically show you the periphery of the biopsy specimen,you can bet it will be myositis ossificans because that is where thecharacteristic features of trabecular osteoid, rimming osteoblasts and remnantsof the muscle tissue will be found. The osteoscarcoma would have the osteoidin the center and be much more disorganized. So once youve figured out this isMO, you know to just watch it. If you excise it, it will often recur, so dont bother.There is nothing to stage, and no adjuvant therapy is recommended for thisbenign process.Enzinger FH, Weiss, SW: Osseous soft tissue tumors, in Enzinger, FM, WeissSW (eds): Soft Tissue Tumors, ed 3. St Louis, MO, Mosby, 1995, pp1013-1037.Huvos, AG: Miscellaneous tumors of soft tissue and bone, in Huvos AG (ed):Bone Tumors. Diagnosis, Treatment and Prognosis, ed 2. Philadelphia, PA, WBSaunders, 1991, pp 745-753
    • Question 161 -A 42-year-old woman has had persistent aching pain in her right arm for thepast 9 months that now awakens her from sleep. Management consisting oforal narcotics has failed to provide relief. Figures 39a and 39b show the plainradiographs of the right humerus, and Figure 39c shows the biopsy specimen.What is the most likely diagnosis?1. Chondrosarcoma2. Ewing’s sarcoma3. Enchondroma4. Osteosarcoma5. Malignant fibrous histiocytoma
    • Answer: 1The radiographs show a long bone diaphyseal intramedullary process, maybesome lytic stuff going on inferiorly, calcification in the proximal portion, someerosion into the endosteal surface on the close-up, no real periosteal reaction,and no cortical expansion- The micrograph looks kind of like cartilage, with cellsand matrix, and the cells dont look too nasty- dont see giant cells ormultinucleated cells all over the place. The other key feature is the process ispainful. This is all typical of chondrosarcomas. Ewings typically have periostealreaction, and the biopsy should be more cellular. Enchondromas are typicallynot painful, they dont erode endosteal surfaces, and the biopsy would be lesscellular. The radiograph doesnt look anything like an osteosarcoma- notaggressive enough, no periosteal reaction, etc. MFH typically wont have thelytic areas, and the micro would be very different- much more cellular, lots ofatypia, giant cells.Unni KK: Chondrosarcoma (primary, secondary, dedifferentiated, and clearcell), in Unni KK (ed): Dahlins Bone Tumors: General Aspects and Data on11,087 cases, ed 5. Philadelphia, PA, Lippincott-Raven 1996, pp 71-108.Wold LE, McLeod RA, Sim FH, Unni KK (eds): Atlas of Orthopedic Pathology.Philadelphia, PA, WB Saunders 1990, Ch 3, 10, 15, 23, 35.
    • Question 166 -Figures 41a through 41c show the axial T1- and T2-weighted images and thebiopsy specimen of a 53-year-old patient who has a painless mass of the leftproximal thigh. A radiograph and CT scan of the chest and a bone scan arenormal. Management should consist of1. radiation therapy and chemotherapy2. radiation therapy and wide excision3. radiation therapy and debulking4. wide excision alone5. marginal excision
    • Answer: 2Using the TNM staging system, tumors are staged as I (low-grade G1), stage II(intermediate), stage III (high grade), and any tumor with spread (mets) is StageIV. Using the AJCC system: tumors < 5 cm are T1, those > 5 cm are T2; Anypatient with a soft tissue mass should receive a CXR to initially screen. Bonescans or CT’s are used to detect metastases. Biopsy specimens are used todetermine the type of soft tissue mass. This biopsy revealed fibroblastic type ofnuclei, nuclei of various shapes and sizes, and occasional mitotic figures. Thescreening exams were negative for metastasis. The MRI revealed the mass tobe fibrous (~ muscle on T1, bright on T2). Radiation therapy (followed by wideexcision) is necessary for large tumors to improve chances for local control.Surgery is the mainstay for treating soft tissue sarcomas. Radiation therapycan be done either pre-operatively or post-operatively, however, pre-operativelyit will shrink the tumor mass, and often cause the formation of a pseudocapsulearound the mass, helping to further isolate/localize the tumor and reduce theamount of tissue resected (permitting limb-sparing surgery).Chang, et al: Clinical evaluation and treatment of soft tissue tumors, inEnzinger and Weiss: Soft-Tissue Tumors, ed 3., 1995, pp 17-38
    • Question 176 -Figure 45 shows the AP radiograph of the pelvis of a 42-year-old woman whoreports the insidious onset of anterior pelvic pain over the past 2 years. Historyreveals that she had a hysterectomy 7 years ago. Examination revealslocalized tenderness over the symphysis pubis, and laboratory studies show anerythrocyte sedimentation rate of 2 mm/hr (normal up to 20 mm/hr).Microscopic examination of the involved area would most likely reveal1. osteomyelitis.2. osteomalacia.3. osteonecrosis.4. neoplasia.5. chronic inflammation.
    • Answer: 55 Ref. JBJS 1989;71A:358-364Osteitis pubis is a painful, inflammatory, but non-infectious condition involving theperiosteum, bone, cartilage, and ligamentous structures in the region of the symphysispubis (2,7,28). Because of the variety of pathological changes with which osteitis pubismay be associated, there has been considerable confusion in the literature as to theprecise cause of the disease. In 1924, Beer{3 } first described osteitis pubis, whichdeveloped after urological procedures. Since that time, numerous cases have beenreported in association with pregnancy, rheumatological disorders, trauma, infection,and athletic exertion, as well as after urological and gynecological operations.In the referenced article on patients with osteitis pubis: all had localized tenderness topalpation over the symphysis pubis, and anteroposterior radiographs, made with thepatient standing, showed the bilateral involvement of the symphysis pubis that ischaracteristic of osteitis pubis. In the four patients who had been first seen less than sixmonths after the onset of symptoms, the radiographs showed rarefaction and erosion ofthe adjacent pubic bones forming the symphysis and widening of the symphyseal jointspace . In the six patients who were first seen more than six months after the onset ofsymptoms, the radiographs showed sclerosis of the adjacent pubic bones and narrowingof the symphyseal joint space.
    • Question 177 -A 72-year-old man reports a progressive increase in pain in his right hip, wherehe has had very mild aching pain for several years. Figure 46a shows the plainAP radiograph of the pelvis, and Figures 46b and 46c show the coronal T1- andT2-weighed MRI scans. A biopsy specimen from the femoral neck region isshown in Figure 46d. What is the most likely diagnosis?1. Dedifferentiated chondrosarcoma2. Telangiectatic osteosarcoma3. Pagetoid osteosarcoma4. Metastatic carcinoma5. Bone infarct-associated osteosarcoma
    • Answer: 3Explanation: AP radiograph and MRI of the pelvis show a lytic lesion involving the entire Right femoral head andneck. Biopsy specimen shows a highly cellular bony specimen with irregular thickened bony trabeculae. Thebone marrow is replaced by vascular fibrous tissue with very irregular appearing cells. This specimen showssome evidence of the classic mosaic pattern of Pagetoid bone.Sarcomatous transformation in Paget’s disease occurs in 0.9% to 5.5% of cases, most commonly in Males (2 : 1),with an average age of 67 (42-78). Most commonly involving the pelvis, femur, humerus, and skull with mostlesions occurring in the metaphyseal-diaphyseal region. These patients typically present with acute pain or aprogressive increase in the intensity of chronic pain and swelling or associated soft tissue mass. Pathologicfracture is the initial presentation in 10-37% of the cases AP radiograph and MRI typically show a lytic lesion withirregular margins and cortical penetration often with an associated soft tissue mass. Biopsy, which is required fordefinitive diagnosis, shows bony trabeculae with the typical mosaic pattern and enlarged hyperchromic andirregular nucleated cells. Multinucleated giant cells are also typically seen.Telangiectatic osteosarcoma – Typically occurs in the 2nd decade of life. Radiographs show purely lytic lesion withno sclerosis. Biopsy of the lesion shows minimal osteoid production with spaces separated by Septa as seen inan aneurysmal bone cyst.Bone infarct-associated osteosarcoma – Typically occurs in the 1st or 2nd decade, and presents with acute onsetof pain and swelling. The roentgenograms typically show a well-circumscribed lesion with mineralization at theperiphery and irregularly mineralized soft tissue masses. Biopsy shows minimal osteoid production.Dedifferentiated chondrosarcoma – Radiographs show dense mineralization surrounded by poorly defined lyticlesions. Biopsy shows hyaline cartilage surrounded by cellular neoplasm.Metastatic carcinoma – Radiographs demonstrate irregular destructive lesions, mostly osteolytic with the biopsyshowing glandular arrangements of epithelial cells in the stroma. Patients may have a know primary cancer andoften present with systemic signs and symptoms of metastasis.References: Hadjipavlou A, Lander P, Srolovitz, et al: Malignant transformation in Paget’s disease of Bone.Cancer 1992;70:2802-2808.Unni KK; Osteosarcoma, in Unni KK (ed): Dahlin’s Bone Tumors: General Aspects and Data on 11,087 cases, ed5. Philadelphia, PA, Lippincott-Raven, 1996, pp 143-183.
    • Question 188A 12-year-old girl sustained several puncture wounds to her palm from catbites. Twenty-four hours later the hand is swollen, erythematous, and painful.What organism is most likely associated with this infection?1. Eikenella corrodens2. Proteus mirabilis3. Streptococcus4. Staphylococcus aureus5. Pasteurella multocida
    • Answer: 5The acute onset of cellulitis, lymphangitis, and serosanguineous or purulentdrainage from hand wounds 12-24 hours after cat or dog bites should suggestPasteurella multocida. P. multocida has been isolated in oral cavities of up to70% of domestic cats and 66% of domestic dogs.The oral drug of choice for animal bites is Augmentin.Reference: Aron MS, Fernando L: Pasteurella multocida: The major cause ofhand infections following domestic animal bites. Lucas GL, Bartlett DH: Pasteurella multocida infection of the hand. PlatReconstr Surg 1981; 67:49-53.
    • Question 189 -Which of the following is considered the most common presenting symptom in apatient with a high-grade primary malignant bone tumor?1. Weight loss2. Fatigue3. Fever4. Pain5. Malaise
    • Answer: 4 The most common presenting symptom in people with high gradeprimary malignant bone tumor is pain. Weight loss, fatigue, fever and malaiseare symptoms seen with malignancy. However they are usually not thepresenting symptom encountered in primary malignant bone tumor.Source: McCarthy EF, Frassica FJ (eds): Pathology of Bone and Joint Disorderwith Clinical and Radiological Correlation. Philadelphia, PA, WB Saunders,1998, p197.
    • Question 193 -Which of the following organisms can produce gas gangrene and maynecessitate open amputation above the level of infection?1. Pseudomonas aeruginosa2. Staphylococcus aureus methicillin-resistant3. Staphylococcus aureus coagulase-negative4. Clostridium perfringens5. Group D enterococcus
    • Answer: 4 DeHaven KE, Evarts CM: The continuing problem of gas gangrene: A reviewand report of illustrative cases. J Trauma 1971;11:983-991. MacLennan has defined gas gangrene as an invasive, anaerobic infection ofmuscle, characterized by extensive local edema, massive tissue necrosis, variabledegrees of gas production, and profound toxemia. The bacteriology of his condition has been well studied. There are six speciesof clostridia capable of producing gas gangrene (table 1). The most important of which isC. perfringens. These organisms are Gram-positive rods that seldom produce spores inthe tissues or in culture media. They are obligate anaerobes, and cannot multiply in therelatively high oxidation-reduction potentials found in healthy tissues. Clostridia arewidely distributed in nature, being found in soil and in feces of most animals, includingman.BacteriologyA. Organisms B. ToxinsClostridium perfringens (C. welchii) Lecthinase ( toxin )C. novyi CollagenaseC. septicum HyaluronidasC. histolyticum LeukocidinC. bifermentans DeoxyribonucleaseC. fallax Protease Lipase
    • Laggaard SW, McElfresh EC,Premer RF: Gangrene of the upper extremity indiabetic patients. J Bone Joint Surg 1989;71A:257-264.Twenty-two patients who had diabetes mellitus and needed an amputation for gasgangrene in an upper extremity at an age of fifty-one years were identified andfollowed…Oe patient was not included in the study because the gangrene had been causedby an aggressive infection with Clostridium perfringens…Gangrene was thought to have developed spontaneously in seven upperextremities, as the patients were unable to recall an injury or infection that precededthe gangrene. Seven patients associated the development of gangrene with anepisode of minor trauma: a cracked nail in two patients and a sliver, blister, fall,bump, and blow from a hammer in one patient each. The gangrene was precededby spontaneous infection in eight patients, although the infecting organisms, whichwere always multiple, were identified in preoperative or intra-operative cultures inonly four of these eight patients. The organisms that were identified as an initialinfection were Staphylococcus (3 infections), Streptococcus (2 infections, Proteus(2 infections), Enterobacter (1 infection) and Veillonella (1 infection). In patientswho did not have an initial infection, the cultures of secondary infections grewStaphylococcus twice, Proteus once, and Corynebacteria once.The results of amputation of the upper extremity were very unsatisfactory. In onlytwo of the twenty-two patients did the surgical wound heal at the level that wasinitially selected, without additional surgical intervention…
    • Question 200 -Which of the following soft-tissue sarcomas most commonly shows prominentmineralization within the lesion on plain radiographs?1. Rhabdomyosarcoma2. Liposarcoma3. Epithelioid sarcoma4. Synovial sarcoma5. Angiosarcoma
    • Answer: 4 ―Amorphous calcifications are frequently present (up to 30% ofpatients) in synovial sarcomas and thus this entity should always be consideredin the differential diagnosis of a soft tissue mass that contains amorphouscalcification.‖ Calcification can be observed in benign soft tissue lesionsincluding nodular (pseudosarcomatous) fasciitis and myositis ossificans.
    • Question 207 -Figures 55a through 55c show the AP radiograph and T1- and T2-weighted axialMRI scans of an 8-year-old boy who has a painless mass in the rightantecubital space. The mass increases in size with activity and then decreasesafter activity. Gross blood is obtained during a needle biopsy. Managementshould include1. wide resection.2. observation.3. open incisional biopsy.4. CT scan-guided core biopsy.5. radiation therapy.
    • Answer: 2A hemangioma may be present at birth or may exist initially as a small red spot.Within several months these lesions undergo rapid growth and proliferation andusually reach maximal size by two to three years of age. Lesions within thehand and wrist are usually circumscribed and have no predominant location. Byage five, spontaneous involution is underway. This process is heralded by grayareas of lighter pigmentation called "herald spots." Most of these lesions havethe appearance of a "strawberry" if dermis is involved, but many deeper lesionswill show few skin changes. CT scan will demonstrate hemangiomas to behomogeneous lesions with well-defined borders. MRI demonstrates depth ofstructures as well as distinguishing these lesions from sarcomas and capillarymalformations. Treatment is conservative. Compression garments for largelesions and reassurance of parents remains the treatment of choice. Earliersurgery is reserved for serious obstructions of the aerodigestive tract,obstruction of vision, gastrointestinal bleeding, and other life-threateningconditions. Early surgery is rarely indicated in the upper extremity.OKU: Hand Surgery Update, Chapter 36: Pediatric Hand Tumors
    • Question 212Which of the following antibiotics is contraindicated in patients who require oralanticoagulants?1.Trimethoprim2.Clindamycin3.Ciprofloxin4.Aminoglycoside5.Erythromycin
    • Answer: 5 Erythromycin interacts with oral anticoagulants to increase prothrombintime. Sulfamethoxazole, Fluconazole, Metronidazole and Rifampin alsoincrease prothrombin time. Trimethoprim combined with methotrexate isassociated with megaloblastic pancytopenia. Clindamycin enhances effects ofneuromuscular blocking agents. Ciprofloxacin increases theophyline levelswhile its absorption is decreased by antacids, iron and zinc salts, and Carafate.Aminoglycoside’s nephro- and ototoxicity is enhanced when combined withdiuretics. Aminoglycides enhance respiratory suppression of neuromuscularblocking agents.Reference: Kasser JR (ed): Orthopaedic Knowledge Update 5. Rosemont, IL,American Academy of Orthopaedic Surgeons, 1996, ppp149-161
    • Question 235 -Figures 59a through 59c show the radiograph and biopsy specimens of an11-year-old child who has distal thigh pain. What is the most likely diagnosis?1- Nonossifying fibroma2- Aneurysmal bone cyst3- Intraosseous ganglion4- Periosteal chondroma5- Telangiectatic osteosarcoma
    • Answer: 2An aneurysmal bone cyst is a solitary, expansile lesion of unknown etiologywhich is generally eccentric in location. These lesions are most commonly seenin individuals under 20 years of age, where swelling, pain, and/or tendernessmay be the presenting complaint. The periphery of the lesion is often indistinct,and the tumor itself often has a trabeculated appearance. On histologicexamination, the lesion is found to contain cystic spaces of different sizes whichare filled with blood but are not lined with a vascular endothelium. Between theblood filled spaces are fibrous septa containing giant cells and foci of immaturebone or osteoid.Bullough, PG: Orthopaedic Pathology, ed 3. Chicago, IL, Mosby-Wolfe, 1997,pp. 402-404.
    • Question 243A 12-year-old boy has pain at rest and at night over the proximal tibia. Plainradiographs show a 6mm lucent area in the cortex surrounded by scleroticbone. What is the most likely diagnosis?1. High-grade osteosarcoma2. Periosteal osteosarcoma3. Osteoblastoma4. Osteoid osteoma5. osteofibrous dysplasia
    • Answer: 4 Osteoid osteoma is a benign tumor of bone that affects 5-20-year-olds.It has a male to female ratio of 3/1. Patients complain of night pain, relievedwith ASA, NSAIDS or prostaglandin inhibitors. X-ray findings are consistentwith the test question, that is a well circumscribed radiolucent nidus that is lessthan 2 cm in diameter. The lesion is typically surrounded by dense reactivebone. The other choices are incorrect, not consistent with the history. Osteoblastoma would have a similar picture on X-ray except thelucency tends to be greater than 2 cm. It also lacks night pain. Periosteal osteosarcoma and high-grade osteosarcoma typicallypresent with a painful, palpable mass, both with distinct X-ray findings. Osteofibrous dysplasia usually affects girls less than 10-years-old.Pain is usually absent unless a pathological fracture occurred. May see anenlarged tibia with anterolateral bowing. On X-ray one will see a radiolucent,expansible and often bubbly appearance with well-defined margins in themetadiaphyseal anterior cortex of the tibia.
    • Question 251 -Figures 64a and 64b show the plain frog lateral radiograph and axial MRI scanof a 7-year-old child who has had left groin pain and a limp for the past 3months. A biopsy reveals an aneurysmal bone cyst. Management shouldinclude1. observation with serial radiographs.2. hip spica casting.3. curettage and bone grafting.4. curettage and cementation.5. en bloc wide excision.
    • Answer: 3 The patient’s plain x-rays show an aneurysmal bone cyst of the leftproximal Femur. In light of the patient’s 3 month history of groin pain and limpfor 3 months, surgical curettage and bone grafting is indicated.Ref: Cancer, 1992; 2921-2931.
    • Question 257 -Figures 65a and 65b show the hematoxin eosin stains of the biopsy specimen,and figures 65c and 65d show the T1 and T2 weighted axial MRI scans of a 36year old woman who has a painless mass in the posterior thigh. Managementshould include:1. marginal excision2. wide excision3. wide excision and radiation therapy4. radiation therapy alone5. amputation
    • Answer: 1 Lesion in figures are most likely representative of neurilemmomaswhich are most commonly found in extremities. These lesions are usuallytreated by excision with preservation of nerve and major trunk.
    • Question 267Figures 71a and 71b show the plain AP radiograph and coronal T1-weightedMRI scan of a 75 year old patient who has had progressively worsening pain inthe left hip for the past 6 months. Figure 71c shows the incisional biopsyspecimen. Staging studies show localized disease. Surgical treatment shouldinclude:1. prophylactic fixation2. curettage and cementation3. curettage and bone grafting4. en bloc wide excision5. hemipelvectomy
    • Answer: 4The plain films, MRI, and biopsy clearly show a chondrosarcoma.―Low grade peripheral lesions (chondrosarcoma) have almost no risk ofmetastasis and an extremely low recurrence rate when a wide margin isachieved‖Reference: Musculoskeletal Tumor Surgery. 1983. p 963-4. Enneking, WF,ed. Churchill Livingstone, New York, NY
    • OITE 200014. An otherwise healthy 10-year-old boy has an erythematous, painful epitrochlear lymph node after visiting a relative with cats 3 days ago. Which of the following organisms is the most likely cause of this problem? 1- Bartonella henselae 2- Mycobacterium marinum 3- Eikenella corrodens 4- Blastomycosis dermatitidis 5- Pasteurella
    • Answer: 1Bartonella henselae is one of four Bartonella species that have been shown to be pathogenic for humans. B. henselae isthe cause of cat-scratch fever, a localized lymphadenopathy in a person who reports being in contact with or beingscratched by a cat. The pathologic response is generally a granulomatous, suppurative, extracellular and intracellular one,self-limited, mild and usually unresponsive to antibiotic treatment. The diagnosis is made by biopsy of the involvedlymph node and a positive skin test with sterile pus from the biopsy.The orthopaedic reference (Laskin and Potenza) presents two cases of cat-scratchfever that presented as a soft tissue mass in an otherwise healthy child. A tentative diagnosis of an atypical soft tissuemass was entertained, but open biopsy confirmed an infected, granulomatous process. Thus, an infectious process mustalways be entertained in the differential diagnosis of a soft tissue mass work-up.Mycobacterium marinum causes ―swimming pool granuloma,‖ ulcerating, granulomatous lesions that occur in the skin atthe site of abrasions incurred at swimming pools. The natural habitat is both fresh and salt water, with the treatmentbeing tetracycline.Eikenella corrodens is a gram-negative rod found in the human mouth flora. Although not the most common organism(Alpha-hemolytic strept. and S. aureus are the most common) isolated from fight bite injuries (7-29%), it must becovered in the antibiotic regimen (i.e. Augmentin).Blastomycosis dermatitidis is a mold in the soil, endemic in North and Central America causing respiratory tractinfections, but is usually asymptomatic and rarely recognized. Disseminated disease may result in ulcerative granulomasof skin, bone or other sites, with Ketoconazole the drug of choice. Pasturella multocida is a gram-negative rod found inthe mouth flora of dogs and cats. About 26% of animal bites become infected with the organism (alpha-hemolytic streptmost common 46%). Penicillin or augmentin remains the antibiotic of choice.References:Bass JW, Vincent JM, and Person DA. The expanding spectrum of Bartonella infections: II. Cat-scratch disease. PediatrInfect. Dis J. 1997: Feb;16(2):pp 163-79.Laskin RS and Potenza AD. Cat Scratch Fever—a confusing diagnosis for the orthopaedic surgeon. JBJS 1971; Vol 53-A, no.6, Sept. pp.1211-1214.
    • 33. Improvement in hip range of motion following cemented total hip arthroplasty in patients with ankylosing spondylitis has been found to be limited by 1- infection. 2- neurologic involvement. 3- heterotopic ossification. 4- soft-tissue contractures. 5- ankylosis of the lumbar spine.
    • Answer: 3Arthritis of the hip joint occurs in approximately 30% of patients with ankylosingspondylitis and, when present, it is usually bilateral. Cemented total hip arthroplasty hasbeen shown to provide excellent relief of pain and improved ambulatory capacity.However, the improvement in the total range of motion of the hip was limited in severalseries due to the high incidence of heterotopic ossification. In one series of 29 hips, severeheterotopic ossification occurred in 23% of the hips. Prophylaxis against heterotopicossification by means of either low-dose radiation or indomethacin therapy is generallyrecommended.References:OKU: Hip and Knee Reconstruction, 1996, pp 79-86.
    • 46. Which of the following processes is related to osteofibrous dysplasia (Campanaccis disease)? 1- Nonossifying fibroma 2- Osteoid osteoma 3- Adamantinoma 4- Fibrosarcoma 5- Olliers disease (multiple enchondromatosis)
    • Answer: 3 Osteofibrous dysplasia and adamantinoma are uncommon lesions that usually occur in thetibia. They were considered unrelated until the authors of recent publications suggestedthat osteofibrous dysplasia is either the benign counterpart of a neoplastic process thatproduces adamantinoma or that it is the residual of a spontaneously regressingadamantinoma. Alternate possibilities include osteofibrous dysplasia is a precursor to andmay become adamantinoma, this, based on a few isolated cases of osteofibrous dysplasiaapparently becoming adamantinoma. The importance of understanding their relationshipis significant because observation is the recommended treatment for a patient withosteofibrous dysplasia.References:Springfield DS, Rosenberg AE, Mankin HJ, Mindell ER,: Relationship betweenosteofibrous dysplasia and adamantinoma. Clin Orthop 1994; 309:234-244.Bridge JA, Dembinoski A, DeBoer J, Travis J, Neff JR: Clonal chromosomal abnormalitiesin osteofibrous dysplasia: Implications for histopathogenesis and its relationship withadamantinoma. Cancer 1994;73:1746-1752.
    • 54. Which of the following soft-tissue lesions is best described as a Musculoskeletal Tumor Society stage 3 lesion (aggressive)? 1- Nodular fasciitis 2- Lipoma 3- Malignant fibrous histiocytoma 4- Fibromatosis (extra-abdominal desmoid) 5- Giant cell tumor of the tendon sheath
    • Answer: 4MSTS stage 3 lesion is a benign aggressive lesion. Therefore one can exclude malignantfibrous histiocytoma. A giant cell tumor is an aggressive lesion but not a giant cell tumorof the tendon sheath. An extra-abdominal desmoid is the most locally invasive of allbenign soft tissue tumors. The lesion infiltrates adjacent tissues. Therefore, it is theanswer to this question.
    • 79. A 28-year-old woman has had intermittent aching pain in the left ankle for the past year that is exacerbated by activity. Figures 17a through 17e show the plain radiograph, the coronal T1-weighted MRI scan, the axial T2-weighted MRI scan, and low- and high-power photomicrographs. What is the most likely diagnosis? 1- Giant cell tumor 2- Osteochondroma 3- Enchondroma 4- Chondromyxoid fibroma 5- Chondroblastoma
    • Answer: 4Chondromyxoid fibroma usually presents as an eccentrically placed lytic lesion with welldefined margins in the metaphysis of the lower extremity. The lesion usually has a scleroticmargin of bone. Usually presents in the 2-3 decade. These are all clues to the diagnosis,but as Dr. Mott says the proof is in the pudding, and the histology gives the diagnosis.Histologically, nodules of cartilage are found between nests of fibromyxoid areas. Thechondrocytes are often spindle shaped.References:Scarborough MT, Moreau G: Benign cartilage tumors. Orthop Clin North Am1996;27:583-589.
    • 82. A 10-year-old girl has had knee pain for the past 3 months. History reveals that an incidental knee radiograph obtained 2 years ago showed no skeletal abnormalities. Current plain radiographs and a biopsy specimen are shown in Figures 18a through 18c. The patients current condition is most likely associated with 1- familial infantile retinoblastoma. 2- multiple hereditary osteochondromatosis. 3- multiple enchondromatosis. 4- polyostotic fibrous dysplasia. 5- Gauchers disease.
    • Answer: 1Retinoblastoma gene mutation is associated with development of osteosarcoma. Theradiographs are consistent with osteosarcoma. The plain films show an aggressive lesionin the distal femur. As Dr. Mott says all lesions around the knee in the 2nd decade of life isosteosarcoma until proven otherwise. Histology confirms the diagnosis ass it showstumor cells producing osteoid. A two step answer as 1st recognize the osteosarcomadiagnosis, then make the connection to retinoblastoma. RB gene is a tumor suppressorgene and when both alleles mutate, thus decreases tumor suppression and makes thesubject more susceptible to oncologic sequelae.References: Hansen MF: Molecular genetic consideration in osteosarcoma. Clin Orthop1991;270:237-246.
    • 87. A 62-year-old man has had an enlarging painless mass on his thigh for the past 4 months. A biopsy specimen and MRI scans are shown in Figures 19a through 19c. This type of tumor will most often metastasize to which of the following structures? 1- Bone 2- Lungs 3- Liver 4- Lymph nodes 5- Kidney
    • Answer: 2Soft tissue tumors metastasize to the lungs. You don’t really even need to look at the MRI andphotomicrograph. You don’t need to waste any precious brain cells figuring out which type oftumor it is, except to realize it is big (more than 5cm), and since it is rapidly growing it will bemalignant. I couldn’t find the soft tissue tumor book they referenced, but another tumor book byEnzinger and Weiss tells us that ―by far the most frequent site of metastasis is the lungs‖. (page 22)There are two series in the journal Cancer that have 26/36 (72%) and 69/77 (90%) of MFH metsshow up in the lungs. (Bertoni, 1985 and Pezzi, 1992) The lymph node answer is the one to trip youup, but most of the tumors we will be faced with don’t do nodal mets as much as pulmonary.(Epithelioid sarcomas, embryonal rhabdomyosarcomas, angiosarcs and melanoma do nodes- youknow enough from the pictures that the tumor isn’t one of those. Just remember lungs for allortho tumor mets and you’ll be safe.References:Chang AE, Sondak VK: Clinical Evaluation and Treatment of Soft Tissue Tumors (ch 2) andMalignant Fibrous Histiocytoma (ch 15), in Enzinger FM and Weiss SW (eds): Soft Tissue Tumors,3rd ed. St. Louis, Mosby, 1995, pp17-23 and pp351-69.Bertoni, F, Capanna R, Biagini R, Bacchini P, et al, Malignant Fibrous Histiocytoma of Soft Tissue.Cancer 1985; 56:356-67.Pezzi CM, Rawlings MS, Esgro JJ, Pollock RE, Romsdahl MM, Prognostic Factors in 227 Patientswith Malignant Fibrous Histiocytoma. Cancer 1992; 69:2098-2103.
    • 92. A 52-year-old woman with no history of malignancy has had mild aching pain in the left tibia for the past 2 years. Examination reveals a firm mass in the subcutaneous border of the tibia; a bone scan shows this to be an isolated finding. Figures 20a through 20d show the plain radiograph, the sagittal Tl-weighted MRI scan, the axial T2-weighted MRI scan, and the biopsy specimen. Management should now include 1- serial radiographic observation. 2- curettage and bone grafting. 3- wide en bloc excision. 4- chemotherapy and wide en bloc excision. 5- prophylactic stabilization and radiation therapy.
    • Answer: 3Whenever I hear about adamantinoma I think of Adam and the Ants, the punk rock groupfrom the1980’s. Anyway. Must differentiate OFD / fibrous dysplasia / adamantinoma.Adamantinoma is treated with wide en bloc excision. Mets can occur in up to 30 percentof cases which can lead to death (tends to occur in cases where incomplete resections wereperformed). Does not respond to chemotherapy or radiation therapy.OFD treatment is close observation. After the age of ten, the lesion should be biopsiedand extensive histological study should be done to exclude the presence of epitheloidcomponents, even if the radiological findings are highly suggestive for OFD. Largespecimens need to be taken at biopsy to avoid missing tumour cells (no needle biopsy).
    • 93. Normal cortical bone has which of the following MR signal characteristics?1- Low on T1-weighted images and low on T2-weighted images2- Low on T1-weighted images and high on T2-weighted images3- Moderate on T1-weighted images and low on T2-weighted images4- High on T1-weighted images and low on T2-weighted images5- High on Tl-weighted images and high on T2-weighted images
    • Answer: 1The following is the chart that needs to be memorized!Tissue T1-weighted T2-weightedFat high intermediate to highArticular cartilage intermediate highFluid (joint, CSF) low highCortical bone low lowTendon/ligament low low /fibrocartilageHemosiderin low very lowReferences:Beaty JH (ed): Orthopedic Knowledge Update 5. Rosemont, IL, American Academy ofOrthopedic Surgeons, 1999, pp 81-87
    • 106. Figure 28 shows the AP radiograph of a 65-year-old woman who has mild shoulder pain and anemia. History reveals no significant illnesses or injuries. What is the next step in evaluation? 1- MRI scan 2- Serum protein electrophoresis 3- Serum alkaline phosphatase studies 4- Open biopsy 5- Needle biopsy
    • Answer: 2This patient has radiographs consistent with a diagnosis of multiple myeloma. It is aneoplasm composed of plasma cells showing various degrees of differentiation. Theprocess is usually multi-centric and diffusely involves the bone marrow. Increasing pain isthe most common patient complaint, and is most often centered in the thoraco-lumbarspine. Most patients will also complain of weakness and weight loss. Pathologic fractureis common, again most commonly in the vertebral column. Diagnosis is confirmed byserum electrophoresis revealing a single monoclonal protein spike. The presence ofBence-Jones proteins in the urine and moderate to severe anemia are other commonfindings. Radiographically, these patients demonstrate multiple osteolytic, ―punched out‖lesions throughout the axial and appendicular skeletons. Bone scan is not helpful as theselesions appear cold secondary to a predominance of osteoclastic activity. An MRI wouldbe superfluous; this is a plain film diagnosis. Biopsy is not indicated. Serum alkalinephosphatase elevation may be present in multiple neoplastic conditions, but notspecifically in multiple myeloma.References:Unni KK: Dahlin’s Bone Tumors, ed 5, Philadelphia, PA, Lippincott-Raven, 1996, pp225-236.
    • 111. Which of the following bone tumors is typically multifocal and involves bones in the same extremity? 1- Osteoblastoma 2- Osteosarcoma 3- Chondrosarcoma 4- Chondroblastoma 5- Hemangioendothelioma
    • Answer: 5Hemangioendothelioma (Hemangiosarcoma) is a rare malignant vascular tumor of bone. Up to 1/3of these patients will have multifocal lesions, which are predominantly lytic. This lesion can occur inany age group and usually presents with pain. Osteoblastoma is a benign bone-producing tumor,typically appearing in the spine, hip and proximal humerus. Radiographically, the bone destructionappears moth-eaten. Osteosarcoma is a malignant spindle cell neoplasm that produces osteoid.There are several variants of osteosarcoma including: intramedullary (classic), parosteal, periosteal,telangiectatic, occurring with Paget’s, and post irradiation. The most common is high-gradeintramedullary osteosarcoma, which presents with pain about the knee of a child or young adult. Ofthese, 90% present with a stage IIB lesion (i.e. high-grade extra-compartmental, no metastases) and10-20% have pulmonary metastases. Chondrosarcoma is malignant neoplasm of cartilage occurringin adults and older age groups. They usually present with pain or a mass in the knee, spine, shoulderand pelvis girdles. Chondroblastoma is a benign cartilage tumor centered in the epiphysis, triradiatecartilage of the pelvis and apophysis of young people (i.e. open physes). These patients also presentwith pain.References:Dorfman HD, Czerfniak B: Bone Tumors. St. Louis, MO, Mosby, 1998, pp 369-370.McCarthy EF, Frassica FJ: Pathology of Bone and Joint Disorders. Philadelphia, PA, WB Saunders,1998, p 267.
    • 120. Figure 33 shows the AP radiograph of the femur of an asymptomatic 14-year-old patient. What is the most likely diagnosis? 1- Olliers disease 2- Pagets disease 3- Fracture malunion 4- Residual proximal femoral focal deficiency 5- Fibrous dysplasia
    • Answer: 5Figure 33: AP radiograph of proximal femur with large ground glass lesion extending distal tofemoral neck. It has a well defined rim of sclerotic bone. There appears to be a slight varus bowingto the proximal femur possibly developing into a "Shepards Crook Deformity."This is a radiographic diagnosis, in this case Fibrous Dysplasia. Fibrous Dysplasia is a developmentalabnormality of bone which is either mono or polyostotic. When associated with skin (yellow orbrown patches) and endocrine abnormalities (precocious puberty) in addition to multiple bonelesions the diagnosis of McCune-Albright syndrome can be made. Patients are usually asymptomaticthe lesions being found incidentally on Xray. By far the most common location is the proximalfemure. Radiographically Fibrous Dysplasia usually shows well defined zones of rarefaction (highlylytic or ground glass appearing), often surrounded by narrow rim of relatively sclerotic bone. Theselesions may expand and thin the cortex especially in thinner bones. These lesions may contain largeamounts of cartilidge which may show ring-like or dot-like calcification especially around femoralneck. These lesions can appear aggressive and suggesting diagnois of sarcoma.As for the other answers Olliers Disease is associated with multiple enchondromas which have adifferent radiographic appearance, Pagets disease as well is entirely differnet having characteristiccoarse trabeculae, remodeled cortices, and a blastic appearance. Identifying that a lesion is presentwould rule out fracture malunion and residual PFFD as answers.Reference:Unni KK: Dahlins Bone Tumors, Ed 5 1996 pp 355-433
    • 135. Which of the following is considered a risk factor for osteoporosis?1- Obesity2- Mediterranean heredity3- Fair skin and hair4- A history of manual labor5- Late-onset menopause
    • Answer: 3.―Genetic predisposition (individuals who are fair-skinned and small, have hypermobilejoints, are of Northern European ancestry, or have scoliosis), cigarette smoking, andexcessive alcohol intake are other risk factors.‖References:Beatty JH (ed): Orthopaedic Knowledge Update 6. Rosemont, IL, American Academy ofOrthopaedic Surgeons, 1999, p. 153
    • 143. A 76-year-old woman has had generalized muscle weakness and arthralgias for the past 2 years. Radiographs show generalized osteopenia and a pseudofracture (Loosers zone) in the inferomedial aspect of the femoral neck. Laboratory studies show normal serum calcium and hemoglobin levels and a mildly elevated alkaline phosphatase level. What is the most likely diagnosis? 1- Osteomalacia 2- Osteoporosis 3- Multiple myeloma 4- Leukemia 5- Pagets disease of bone
    • Answer: 1Osteomalacia is a metabolic disorder in which there is inadequate mineralization of newly formed osteoid. It canresult from vitamin D deficiency, vitamin D resistance, intestinal malabsorption, acquired or hereditary renaldisorders, intoxication with heavy metal such as aluminum or iron, and other assorted etiologies. The childhoodform of osteomalacia is termed rickets. The diagnosis of osteomalacia often is difficult because patients usuallyhave nonspecific complaints such as muscle weakness of diffuse aches and pains. Radiographic evidence ofosteomalacia often mimics other disorders including osteoporosis. However, the presence of pseudofractures orLooser’s transformation zones is good evidence that some degree of osteomalacia is present. Usual lab finds –elevated alkaline phosphatase, low calcium or low phosphorus levels. Serum assays for vitamin D metabolites helpclarify the abnormality.Pagets disease of bone is characterized by bone resorption and formation, showing areas of wide lamellae andirregular cement lines, which produce the characteristic mosaic pattern appearance of pagetic bone. Two markersare used to follow the course of disease- serum alkaline phosphatase and urinary piridinium cross links.Osteoporosis is a silent disease, without specific laboratory findings. The only radiographic finding can be afracture (most common wedging of vertebrae), or bone loss, which is detected on radiographs only after 30% to50% of the mineral is lost.Multiple Myeloma will present as lytic lesions in bone, and not as Looser’s lines.Leukemia is more common in children, with skeletal involvement in 50% of cases. Diffuse osteopenia is the mostfrequent manifestation. Sometimes lucencies and periostitis may mimic osteomyelitis.References:Beaty JH (ed): Orthopedic Knowledge Update 5. Rosemont, IL, American Academy of Orthopedic Surgeons,1999, pp427-439.
    • 150. What type of chondrosarcoma has the lowest 5-year disease-free survival rate?1- Chondrosarcoma developing in an osteochondroma2- Chondrosarcoma developing in Olliers disease3- Intermediate grade (grade 11)4- Mesenchymal5- Clear cell
    • Answer: 4Just Memorize.
    • 159. A 21-year-old marathon runner who is 5 2" tall and weighs 95 lb reports the onset of left leg pain when she increased her training program 2 months prior to an event. History reveals that her last menstrual period was 18 months ago. Bone density of the spine tested by DEXA is 1.8 standard deviations below the mean for age. A bone scan is normal. Management should include 1- cessation of running and 10 mg of alendronate per day. 2- nutritional counseling and a stretching program. 3- an ultrasound of the heel and 500 mg of calcium per day. 4- calcitonin, a short leg cast, and multivitamins. 5- evaluation of the amenorrhea, achievement of nutritional balance, and cross- training.
    • Answer: 5Amenorrhea in the female athlete may be related to a low body fat percentage and/orstress. The incidence approaches 50 % in elite runners and is related to stress fx(osteopenia) and eating disorders. Dietary management and birth control pills are helpfulfor treating the problem. A period of cross training would help to alleviatesupraphysiologic stresses and maintain cardiovascular fitness (5 correct). A bone scan failsto show evidence of tibial stress fx so casting is not indicated (4 incorrect).Bisphosphonates are not indicated for this process which should be amendable to dietarymanagement (1 incorrect).References:OKU: Sports Medicine 2 pp 43-47.
    • 171. Normal mineralization of bone is seen in which of the following conditions? 1- Rickets 2- Seizure disorder treated with phenytoin 3- Renal osteodystrophy 4- Fanconi syndrome type II. 5- Osteoporosis
    • Answer: 5Rickets = Osteomalacia in Adults = failure of meneralization due to many causes. Labs arelow normal Ca, low Pa, increased PTH, low Vit D. Many physical deformities result (Millerp31). Tx with Vit D (5000 IU daily) Phenytoin administration is one of the many causes ofrickets. Renal Osteodystrophy is another cause of rickets which is a failure ofmeneralization
    • 173. Which of the following bone lesions arises exclusively in the epiphysis or apophysis of the long bones? 1- Giant cell tumor 2- Aneurysmal bone cyst 3- Unicameral bone cyst 4- Fibrous dysplasia 5- Chondroblastoma
    • Answer: 5Chondroblastomas (and infection) is the only lesion that arises from the epiphysis. Theepiphyseal location is a major clue for making the diagnosis but 52% in the series quoted alsoinvolved the metaphysis. However none of the tumors arising in long bones involved only themetaphysis. The radiographic appearance of chondroblastomas of long bones is relativelytypical when the tumor involves the epiphysis or an apophysis, but radiographic features areless typical for flat bone lesions. The most frequent feature of chondroblastomas in generalwas an eccentric oval or round lytic lesion that was well defined without a sclerotic rim andshowed no sclerosis of the surrounding bone and that eroded or destroyed the adjacent cortexwithout periosteal reaction. A sclerotic rim and matrix calcification are helpful in making thediagnosis , but these features generally are not present. Chondroblastoma can be treatedeffectively with curettage and bone grafting. Giant cell tumors arise in both the epiphyseal andmetaphyseal region of long bones. Aneurysmal bone cysts are located eccentrically in themetaphysis of a long bone and are usually well delineated. The unicameral bone cyst is a benigntumor that develops in metaphyseal bone adjacent to the physis. Fibrous dysplasia frequentlyaffects more that one bone on the same side and is usually metaphyseal.References:Dorfman HD: Bone tumors. St.Louis, MO, Mosby, 1998, pp296-297Turcotter RE: Chondroblastoma. Hum Pathol 1993;23:944-949
    • 183. An 11-year-old boy has an enlarging, slightly tender mass over the proximal phalanx of the third digit. The plain radiograph and a biopsy specimen are shown in Figures 39a and 39b. What is the most likely diagnosis? 1- Giant cell tumor 2- Chondroblastoma 3- Periosteal chondroma 4- Aneurysmal bone cyst 5- Nonossifying fibroma
    • Answer: 3Figure 39a shows a saucer-like erosion with a well-defined rim of reactive bone underlying the lesion which ischaracteristic of periosteal chondroma. Lesions may measure up to 4cm and calcifications may be present. Figure 39bshows lobules of hypercellular, immature cartilage; double nuclei; faint staining matrix and little, if any calcification, andmild cellular atypia which is consistent with this lesion. Periosteal chondromas are benign cartilaginous tumors forming beneath the periosteum and external to the cortex ofbone. This extremely rare lesion, with few cases reported in the literature can cause saucerization of the underlyingcortex and is mainly discovered in the third to fourth decades of life (case reports involve children as young as 4) andarises from subperiosteal cartilage formation. Male to female ration of 2:1. The classic location for this tumor ismetaphyseal cortex of proximal humerus. Surgical treatment consists of resection of symptomatic larger lesions with awide or marginal margin, recurrence with intralesional resection is common. Pain only occurs if the lesion is in proximitywith a joint. Regression with maturity does not occur. Asymptomatic patients with latent lesions may be observed withserial radiographs. Chondroblastoma appear more aggressive radiologically, begin in the epiphyseal portion of long bones, generally arepainful and patients present with associated joint swelling. Histologically this tumor shows immature chondroblasts,giant cells, less cartilaginous matrix and a greater degree of cellular atypia and hypercellular content. ABC’s histologically are composed of spindle cells, some bone formation and large bone cysts that usually are blood-filled and pain is the most common symptom. Giant cell tumors invariably present with pain and are usually located in the proximal tibia and histologically arecomposed of multinucleated giant cells and not cartilage matrix. NOF would not histologically show this immature cartilage matrix.References:Weiner SD, Iorio CD: Painless deformity of a long finger phalanx of a 4-year old girl. CORR 1999;369:357-65.Scarborough MT, Moreau G: Benign cartilage tumors. Orthop Clin North Am 1996;27:583-89.
    • 184. A 30-year-old woman has chronic ankle pain and swelling without any history of trauma. Examination reveals diffuse swelling and soft-tissue fullness along the anterior aspect of the ankle joint. Plain radiographs are normal. An MRI scan shows an effusion of the ankle and a soft-tissue mass arising from the ankle joint that is dark on both Tl- and T2weighted images. A needle biopsy specimen is shown in Figure 40. Management should consist of 1- below-knee amputation and chemotherapy. 2- external beam radiation. 3- extra-articular resection and tibiotalar arthrodesis. 4- open synovectomy. 5- triple antibiotic therapy.
    • Answer: 4Pigmented Villonodular synovitis is probably a proliferative reaction to some type ofinflammatory agent. This reaction, characterized by giant cells, villous and nodular massesthat fuse together in the synovial membrane to form a single mass. Arthroscopy andbiopsy are valuable in making the diagnosis. Figure 40 shows nodules of glandular tissuew/ lipid filles histiocytes and giant cells. PVNS is relatively rare lesion, occurs in adultsand the knee is the usual joint affected. The lesion produces a bulky mass in the synovialmembrane and may even erode bone(this patient c/o mass at anterior ankle and has h/oswelling/inflammation/pain). The lesion in synovial joints responds well to surgicalexcision of the involved area of synovial membrane. For diffuse and widespread intra-articular disease, extensive synovectomy (either open or artroscopic is required.Antibiotics will not help, fusion is not necessary, radiation is not helpful and BKA andchemotherapy is too aggressive for this benign lesion.References:Mizel MS, Miller RA, Scioli MW (eds): Orthopaedic Knowledge Update: Foot and Ankle2. Rosemont IL, American Academy of Orthopaedic Surgeons, 1998, pp11-26.
    • 195. An otherwise healthy 37-year-old man has had bilateral posterior heel pain for the past year. Examination reveals fullness, warmth, and tenderness over the posterior aspect of the heels. Radiographs are normal. Laboratory studies show a normal CBC and an erythrocyte sedimentation rate of 50 mm/h (normal up to 20 mm/h), and an HLA-B27 is positive. What is the most likely diagnosis? 1- Rheumatoid arthritis 2- Ankylosing spondylitis 3- Lupus erythematosus 4- Reiter syndrome 5- Lyme disease
    • Answer: 4Given the clinical presentation (37yo man, bilateral heel pain, elevated ESR, and HLA B-27 +) Reitersyndrome is the logical choice. Reiter’s can consist of the triad of conjunctivitis, urethritis, and arthritis.Unknown cause, 96% + for HLA B-27, lower extremities usually involves knees, ankles, feet. Heel paincan be presenting symptom.Ankylosing Spondylitis - Mainly effects axial skeleton, adolescent males, 90% + for HLA B-27, + hip andBACK pain, XR show bilateral symmetric sacroiliac erosions, followed by joint space narrowing, thenankylosis and late ―bamboo spine‖. Limitation of chest wall expansion is specific for AS.Rheumatoid arthritis - Systemic disease affecting synovial tissues usually symmetrically, 3:1 female to maleratio, 90% have FOREFOOT involvement (hallux valgus, dorsal subluxation of plantar fat pad (walkingon marbles).Lyme disease - spirochete (Borrelia burgdorferi) transmitted by deer tic (Ixodes dammini). Target shapedrash, fever, and systemic disease. Late musculoskeletal overuse syndrome. Endemic regions northeastU.S., Minnesota, Michigan, Oregon and California. Get Lyme titers. High index of suspicion needed. Txwith Zithromax.Lupus erythrematosis - multisystem autoimmune disease, 9:1 female to male ratio, usually 2-3rd decade oflife, skin rashes, joints- nonerosive synovitis with little deformity, renal disorders-glomerular nephritis,hematologic disorders-anemias, neurologic- seizures. Titers of anti-DS DNA and anti-Sm antibodies arevirtually diagnostic of lupus.References:Thomas FM, Mann RA: Arthritides, in Mann RA (ed); Surgery of the Foot and Ankle 1994 pp 618-619.
    • 206. Which of the following diseases is characterized by a defect in type I collagen metabolism? . 1- Diastrophic dwarfism 2- Osteogenesis imperfecta 3- Mucopolysaccharidosis 4- Pseudoachondroplasia 5- Multiple epiphyseal dysplasia
    • Answer: 21-Diastrophic dwarfism: caused by abnormal sulfate transporter leading to undersulfated proteoglycans…proteoglycan swelling is part of the mechanism that produces growth2- Osteogenesis imperfecta: the defect is in the gene for the 1 and 2 chains of type I collagen3- Mucopolysaccharidosis: lysosomal enzyme defect that alters the ability to mobilize glycosaminoglycans (ex. Hunter’s and Hurler’s disease)4- Pseudoachondroplasia: mutation in fibroblast growth factor receptor 35- Multiple epiphyseal dysplasia: autosomal dominant defect in the pericentromeric region of chromosome 19 and 1Reference:Simon SR (ed): Orthopaedic Basic Science. Rosemont, IL, American Academy ofOrthopaedicSurgeons, 1994, pp 187-217.
    • 247. Figure 60 shows the bone mineral density (BMD) report for a 57-year-old Caucasian woman. According to the National Osteoporosis Foundation guidelines, management should consist of 1- observation and serial BMD testing. 2- vitamin D and calcium dietary supplements only. 3- pharmacologic treatment if additional risk factors for fracture are positive. 4- pharmacologic treatment in the absence of additional risk factors. 5- pharmacologic treatment only if the patient has a history of a osteoporotic fracture
    • Answer: 4Physicians Guide to Prevention and Treatment of Osteoporosis: www.nof.org/physguide.Hip BMD is the best predictor of hip fractures and predicts fracture at other sites as well as othermeasurements. BMC is expressed as a relationship between two norms: Z-score: expected BMD for the patient’s age and sex T-score: BMD for a normal, young adult of same sex.The difference between the patients score an Z-score is expressed in the form of standard deviationsabove or below the mean. (one SD = 10-12% difference in bone density). The T-score is theparameter correlated with risk of osteoporotic fracture. Interpretation of T-score is as follows: Above (-1)SD Normal Between (-1)SD and (-2.5)SD Osteopenic Below (-2.5)SD OsteoporoticTreatment: 1) Everyone gets Calcium(1200 mg), Vitamin D(400-800 IU), regular weight bearingand muscle strengthening exercises, and advise to avoid tobacco and to keep alcohol intake to aminimum. 2) Pharmacologic therapy (Hormone replacement, Alendronate, Calcitonin, Raloxifene)is initiated when BMD T-score is <-2 and there are no risk factors or when T-score is <-1.5 andthere are risk factors present.References:National Osteoporosis Foundation Development Committee: Osteoporosis: Physician’s Guide toPrevention and Treatment of Osteoporosis. Belle Mead, NJ, Excerpta Medica, 1998, pp 18-22.
    • 254. What is the rate of bone loss per year at menopause? 1- 0.3% to 0.5% for 3 to 5 years 2- 0.3 % to 0.5 % until age 65 years 3- 2% to 3% for 2 to 3 years 4- 2% to 3% for 6 to 10 years 5- 4% to 5% for 2 to 3 years
    • Answer: 4In OKU 6 and in two supporting papers from the New England Journal of Medicine therate of bone loss per year at menopause is 0.3% to 0.5% for 3 to 5 years.