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Bone Neoplasms Radiographic and Pathologic Correlation

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Bone Neoplasms Radiographic and Pathologic Correlation

  1. 1. Radiographic andRadiographic and Pathologic CharacteristicsPathologic Characteristics of Common Boneof Common Bone NeoplasmsNeoplasms Nick LuemNick Luem Diagnostic RadiologyDiagnostic Radiology June 24, 2007June 24, 2007
  2. 2. Classification of the more commonClassification of the more common bone tumorsbone tumors  Benign NeoplasmsBenign Neoplasms – OsteochondromaOsteochondroma – ChondromaChondroma – Giant Cell TumorGiant Cell Tumor – Aneurysmal Bone CystAneurysmal Bone Cyst  Malignant NeoplasmsMalignant Neoplasms – Osteogenic sarcomaOsteogenic sarcoma – ChondrosarcomaChondrosarcoma – Ewing’s sarcomaEwing’s sarcoma
  3. 3. OsteochondromaOsteochondroma  Also known as exostosisAlso known as exostosis  Children and teenagers most affected, Men >>Children and teenagers most affected, Men >> WomenWomen  Clinically appear as slow growing masses,Clinically appear as slow growing masses, painful if impinging on nerve tissuepainful if impinging on nerve tissue  Solitary or multipleSolitary or multiple  Multiple Hereditary ExostosisMultiple Hereditary Exostosis autosomalautosomal dominant disease with inactivation of bothdominant disease with inactivation of both copies of EXT gene in growth platecopies of EXT gene in growth plate chondrocyteschondrocytes
  4. 4. OsteochondromaOsteochondroma  Benign projection of bone with cartilaginous capBenign projection of bone with cartilaginous cap  Occurs in epiphyseal plate and grows laterallyOccurs in epiphyseal plate and grows laterally  Exhibits cortex and medullary portionExhibits cortex and medullary portion  May convert to malignancy if cartilage capMay convert to malignancy if cartilage cap becomes thicker and contains disorganizedbecomes thicker and contains disorganized calcificationscalcifications  Conversion to sarcoma rare (<1%) but higher inConversion to sarcoma rare (<1%) but higher in patients with hereditary syndromepatients with hereditary syndrome
  5. 5. OsteochondromaOsteochondroma  Ultrasound safe and inexpensive way to evaluateUltrasound safe and inexpensive way to evaluate thickness of cartilaginous capsulethickness of cartilaginous capsule  Develops in bones of endochondral origin and arises from the metaphysis near the growth plate of long tubular bones.  Occasionally develops from bones of the pelvis, scapula and ribs  MRI method of choice to evaluate thicknesss ofMRI method of choice to evaluate thicknesss of cartilaginous cap to rule out malignant conversioncartilaginous cap to rule out malignant conversion
  6. 6. - Radiograph can demonstrate that cortex- Radiograph can demonstrate that cortex of osteochondroma blends with cortex ofof osteochondroma blends with cortex of normal bonenormal bone - Long axis of tumor usually runs parallel- Long axis of tumor usually runs parallel to parent bone and points away fromto parent bone and points away from parent jointparent joint
  7. 7. Osteochondroma: Disorganized growth plate with endochondral ossification Newly made bone forms inner portion of head and stalk Medullary cavity of osteochondroma and bone are continuous
  8. 8. ChondromaChondroma  Slow-growing tumor of hyaline cartilageSlow-growing tumor of hyaline cartilage  EnchondromasEnchondromasarise within medullary cavityarise within medullary cavity  Juxtacortical chondromaJuxtacortical chondromaarise on bone surfacearise on bone surface  Destroys normal bone by erupting as mixture ofDestroys normal bone by erupting as mixture of calcified and uncalcified hyaline cartilagecalcified and uncalcified hyaline cartilage  Occur in children and young adultsOccur in children and young adults  Enchondromas usually solitaryEnchondromas usually solitary  Favor the metaphyseal region of tubular bonesFavor the metaphyseal region of tubular bones such as the small bones of the hand and feetsuch as the small bones of the hand and feet
  9. 9. ChondromaChondroma  Most asymptomatic and found incidentallyMost asymptomatic and found incidentally  Occasionally cause pathologic fracturesOccasionally cause pathologic fractures  Ollier diseaseOllier disease syndrome of multiplesyndrome of multiple enchondromasenchondromas  Maffuci syndromeMaffuci syndromeenchondromatosisenchondromatosis associated with soft tissue hemagiomasassociated with soft tissue hemagiomas  May recur if incompletely excisedMay recur if incompletely excised
  10. 10. -Radiographic findings of enchondromas include a stippled ringlike or arclike calcifications within the lucent matrix -Cartilage nodules can form well-circumscribed oval lucencies surrounded by thin rim of radiodense bone, the “O ring sign” -T2-weighted MR images show lesionT2-weighted MR images show lesion with lobulated borders from endostealwith lobulated borders from endosteal scalloping and containing focal areasscalloping and containing focal areas of high signal intensityof high signal intensity -Nuclear medicine scans usuallyNuclear medicine scans usually negative in enchondromas, rulingnegative in enchondromas, ruling out the possibility of malignancyout the possibility of malignancy
  11. 11. -Nodules of cartilage that are well- circumscribed with a hyaline matrix -The neoplastic chondrocytes in the lacunae are cytologically benign -Cartilage at the periphery of the nodule undergoes endochondral ossification
  12. 12. Giant Cell TumorGiant Cell Tumor  Name derives from abundant multinucleatedName derives from abundant multinucleated osteoclast-type giant cellsosteoclast-type giant cells  Uncommon but locally aggressive tumor. Usually arisesUncommon but locally aggressive tumor. Usually arises in patients in their twenties to forties.in patients in their twenties to forties.  Giant cell tumors in adults involve both epiphyses andGiant cell tumors in adults involve both epiphyses and metaphyses, but in adolescents are confined proximallymetaphyses, but in adolescents are confined proximally by the growth plate and are limited to the metaphysisby the growth plate and are limited to the metaphysis  Majority occur in the distal femur and proximal tibia butMajority occur in the distal femur and proximal tibia but any bone may be involvedany bone may be involved  May cause arthritic symptoms in patients or lead toMay cause arthritic symptoms in patients or lead to pathologic fracturespathologic fractures
  13. 13. Giant Cell TumorGiant Cell Tumor Large red to brown tumors that undergo cystic degeneration
  14. 14. Giant Cell TumorGiant Cell Tumor  Conservative surgery, such as curettage,Conservative surgery, such as curettage, associated with 40% to 60% recurrence rateassociated with 40% to 60% recurrence rate  Up to 4% metastasize to the lungsUp to 4% metastasize to the lungs  Some lesions can be pre-malignant or malignantSome lesions can be pre-malignant or malignant  MRI used to determine intraarticular extension,MRI used to determine intraarticular extension, soft tissue involvement and bone marrowsoft tissue involvement and bone marrow changeschanges  Diagnostic accuracy high when MR images andDiagnostic accuracy high when MR images and X-ray images are combinedX-ray images are combined
  15. 15.  Characteristic radiographic appearance ofCharacteristic radiographic appearance of Giant Cell Tumor: multiple large bubblesGiant Cell Tumor: multiple large bubbles separated by thin strips of boneseparated by thin strips of bone
  16. 16. Giant Cell TumorGiant Cell Tumor Tumor is composed of uniform oval mononuclear cells with indistinct membranes and appear to grow in a syncytium Scattered within this background are numerous osteoclast-type giant cells Necrosis, hemorrhage, hemosiderin deposition and reactive bone formation are frequent secondary features
  17. 17. Aneurysmal Bone CystAneurysmal Bone Cyst  Not a true neoplasm or cystNot a true neoplasm or cyst  Numerous blood filled arteriovenousNumerous blood filled arteriovenous communicationscommunications  Thought to be secondary to traumaThought to be secondary to trauma  Often mistaken for malignant tumor onOften mistaken for malignant tumor on plain radiographplain radiograph
  18. 18. Aneurysmal Bone CystAneurysmal Bone Cyst  CT can show the lobulations of the lesionCT can show the lobulations of the lesion  MRI shows internal loculation and fluidMRI shows internal loculation and fluid levels that produce low signal on T2-levels that produce low signal on T2- weighted imagesweighted images  T1-weighted images show cyst with a lowT1-weighted images show cyst with a low to intermediate signal intensity. Signalto intermediate signal intensity. Signal intensity increases if acute hemorrhageintensity increases if acute hemorrhage presentpresent
  19. 19. Aneurysmal Bone CystAneurysmal Bone Cyst  Expansile, eccentric, cystlikeExpansile, eccentric, cystlike lesion that causes pronouncedlesion that causes pronounced ballooning of thinned cortex inballooning of thinned cortex in long boneslong bones  Cystic lesion has multiple, fineCystic lesion has multiple, fine internal septainternal septa
  20. 20. Aneurysmal Bone CystAneurysmal Bone Cyst Microscopically, the ABC has cysticMicroscopically, the ABC has cystic spaces filled with blood. The fibrousspaces filled with blood. The fibrous septa have immature woven bonesepta have immature woven bone trabeculae as well as capillariestrabeculae as well as capillaries hemosiderin-laden macrophages,hemosiderin-laden macrophages, fibroblasts, and giant cells.fibroblasts, and giant cells.
  21. 21. Malignant neoplasmsMalignant neoplasms  OsteosarcomaOsteosarcoma  ChondrosarcomaChondrosarcoma  Ewing’s SarcomaEwing’s Sarcoma
  22. 22. OsteosarcomaOsteosarcoma  Malignant mesenchymal tumor in which cancerous cellsMalignant mesenchymal tumor in which cancerous cells produce bone matrixproduce bone matrix  Accounts for approximately 20% of primary boneAccounts for approximately 20% of primary bone cancerscancers  Bimodal age distribution; 75% occur in patients <20Bimodal age distribution; 75% occur in patients <20 years. Second peak occurs in older adults who haveyears. Second peak occurs in older adults who have known conditions associated with the development ofknown conditions associated with the development of osteosarcoma (Paget disease, bone infarcts, priorosteosarcoma (Paget disease, bone infarcts, prior irradiation)irradiation)  Metaphyseal region of long bones common site, roughlyMetaphyseal region of long bones common site, roughly 60% occur about the knee60% occur about the knee
  23. 23. OsteosarcomaOsteosarcoma  Patient with hereditary retinoblastomas have up to 1000Patient with hereditary retinoblastomas have up to 1000 times greater risk of developing osteosarcomatimes greater risk of developing osteosarcoma  Attributed to germ line mutations in RB geneAttributed to germ line mutations in RB gene  Abnormalities in other genes that regulate cell cyclingAbnormalities in other genes that regulate cell cycling implicated (CDK4, p16, INK4A, CYCLIN D1, MDM2)implicated (CDK4, p16, INK4A, CYCLIN D1, MDM2)  Typically present as painful and progressively enlargingTypically present as painful and progressively enlarging masses. At time of diagnosis, aprox. 10%-20% ofmasses. At time of diagnosis, aprox. 10%-20% of patients have metastates to the lungspatients have metastates to the lungs
  24. 24. OsteosarcomaOsteosarcoma Reactive periosteal bone forms when tumor breaks through cortex This leaves a triangular shadow between the cortex and raised ends of periosteum known radiographically as “Codman triangle”
  25. 25. OsteosarcomaOsteosarcoma Tan-white tumor fills most of the medullary cavity of the metaphysis and proximal diaphysis The tumor infiltrates through the cortex, lifts the periosteum and forms soft tissue masses on the side of the bone Several subtypes are recognized and grouped by location, degree of differentiation, multicentricity and histologic variance
  26. 26. OsteosarcomaOsteosarcoma Coarse, lacelike pattern of neoplastic bone formed by anaplastic malignant tumor cells. Bone may be deposited in large sheets of primitive trabeculae Cartilage and fibrous tissue may be present in varying amounts. Vascular invasion usually conspicuous and spontaneous necrosis is common
  27. 27. ChondrosarcomaChondrosarcoma Chondrosarcomas produce neoplastic cartilage Second most common malignant matrix-producing tumor of bone Subclassified by: Siteintramedullary, juxtacortical Histologyconventional, clear cell, dedifferentiated, mesenchymal variant
  28. 28. Patients usually >40 yrs of age Men affected twice as frequently as women Commonly arise in central portions of skeleton: pelvis,shoulder and ribs. Clear cell variant originates in the epiphyses of long tubular bones Often contain punctate or amorphous calcification within its cartilaginous matrix Endosteal scalloping and cortical destruction seen radiographically The more radiolucent the tumor, the greater likelihood of a higher grade
  29. 29. ChondrosarcomaChondrosarcoma Tumors vary in degree of cellularity, cytologic atypia and mitotic activity Low grade: mild hypercellularity, plump vesicular nuclei with small nucleoli, and sparse mitotic figures High grade: extreme pleomorphism with bizarre tumor giant cells and mitoses
  30. 30. Ewing’s SarcomaEwing’s Sarcoma  Small round cell tumor of boneSmall round cell tumor of bone  Accounts for 6%-10% of primaryAccounts for 6%-10% of primary malignant bone tumorsmalignant bone tumors  Most patients between 10 to 15 years old;Most patients between 10 to 15 years old; youngest average age at presentation ofyoungest average age at presentation of all bone tumorsall bone tumors  Treatment includes chemotherapy andTreatment includes chemotherapy and surgical excision. At least 50% are curedsurgical excision. At least 50% are cured long termlong term
  31. 31. Classic radiographic appearance: Ill-defined permeative area of bone destruction Involves large central portion of the shaft of a long bone Ewing sarcoma typically arises in the medullary cavity and invades the cortex and periosteum Associated with a fusiform layered periosteal reaction parallel to the shaft, the classic “Onionskin appearance”
  32. 32. Tumor composed of sheets of uniform,small, round cells that are slightly larger than lymphocytes with scant cytoplasm Generally little stroma and necrosis maybe prominent Relatively few mitotic figures in relation to the dense cellularity of the tumor
  33. 33. ReferencesReferences  Robbins and Cotran,Robbins and Cotran, Pathologic Basis of Disease 7Pathologic Basis of Disease 7thth EditionEdition. Elsevier Saunders, Philadelphia, PA. Elsevier Saunders, Philadelphia, PA  Eisenberg,Eisenberg, Comprehensive Radiographic Pathology 4Comprehensive Radiographic Pathology 4thth EditionEdition. Mosby, St. Louis, MO. Mosby, St. Louis, MO  Grainger & Allison’s Diagnostic Radiology: A Textbook ofGrainger & Allison’s Diagnostic Radiology: A Textbook of Medical Imaging 4Medical Imaging 4thth EditionEdition..

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