June 2007 
Tumor Unknown Conference
Case 1 
C.G. - 21 year old male with a history 
of curretage and bone grafting of a 
distal ulnar lesion eighteen months ago
Aneurysmal Bone Cyst (ABC) 
 Osteolytic, hyperplastic, hyperemic-hemorrhagic 
lesion of unknown origin 
 Epidemiology 
 80% before 20 years of age, rare after 30 years old 
 Most common sites: long bones LE > UE 
 30% secondary lesions due to pre-existing tumor 
 Radiographs 
 Eccentric, expansile, lytic lesion of metaphysis 
 Cortical attenuation or destruction 
 Rim of reactive bone 
 Fluid-Fluid levels on MRI are characteristic
ABC 
 Histology 
 Cavernous spaces filled with blood, lacking 
endothelial lining 
 Fibroblastic cells, multinucleated giant cells 
and thin strands of bone
ABC 
 Treatment 
 Excision, curettage, and bone grafting 
 Resection in case of an expendable bone. 
 Other modalities include adjuvant cryo, 
chemical cautery, injection of steroids 
 Radiation not recommended 
 Prognosis 
 Local recurrence rate approx 10%, increased if 
open physis
Case 2 
T.K. - 18 year old male with 
asymptomatic left ankle swelling for 
several months
Aneurysmal Bone Cyst (ABC) 
 Osteolytic, hyperplastic, hyperemic-hemorrhagic 
lesion of unknown origin 
 Epidemiology 
 80% before 20 years of age, rare after 30 years old 
 Most common sites: long bones LE > UE 
 30% secondary lesions due to pre-existing tumor 
 Radiographs 
 Eccentric, expansile, lytic lesion of metaphysis 
 Cortical attenuation or destruction 
 Rim of reactive bone 
 Fluid-Fluid levels on MRI are characteristic
ABC 
 Histology 
 Cavernous spaces filled with blood, lacking 
endothelial lining 
 Fibroblastic cells, multinucleated giant cells 
and thin strands of bone
ABC 
 Treatment 
 Excision, curettage, and bone grafting 
 Resection in case of an expendable bone 
 Other modalities include adjuvant cryo, 
chemical cautery, injection of steroids 
 Radiation not recommended 
 Prognosis 
 Local recurrence rate approx 10%, increased if 
open physis
Case 3 
S.P. - 12 year old male with left hip 
pain after a minor fall
Unicameral Bone Cyst 
 Benign fluid filled cystic lesion of bone 
of unknown cause 
 Epidemiology 
 5-15 years of age 
 Male > Female 
 Proximal humerus most common, followed 
by proximal femur
Unicameral Bone Cyst 
 Symptoms 
 Asymptomatic unless fracture occurs 
 Radiographs 
 Central lytic lesion of metaphysis 
 Attenuation and slight expansion of cortex 
 Fluid filled on CT/MRI
Unicameral Bone Cyst 
 Histology 
 Thin fibrous membrane lining cyst 
 Few macrophages, giant cells, leukocytes 
and slivers of bone and osteoid
Unicameral Bone Cyst 
 Treatment 
 Cysts become inactive at skeletal maturity and 
typically resolve without surgical intervention 
 Multiple treatments including curettage and 
bone grafting, steroid injections have been tried 
with variable efficacy 
 Fractures tend to lead to resolution of cyst 
 Displaced fractures are the only absolute 
surgical indication
Case 4 
A.M. - 87 year old female with an 
asymptomatic posterior thigh soft tissue 
mass present for many years, the patient 
reports that it has increased in size over the 
past three months
Lipoma 
 Benign tumor of mature fat 
 Classification 
 Superficial: (more common) 
 contained within subcutis 
 prevalence for back, neck, proximal limbs 
 Deep: (less common) 
 within muscle or intermuscular spaces 
 Age 
 Most frequent between 40-60 years old 
 Presentation 
 Slow growing asymptomatic mass
Lipoma 
 Imaging 
 Resembles normal fat on all CT and MRI images 
 Histology 
 Mature fat cells (lipocytes) 
 Rarely small areas of hemmorrage, necrosis, calcification 
 Absence of lipoblasts and pleomorphic, hyperchromatic 
nuclei differentiate lipoma from liposarcoma 
 Treatment 
 Marginal Excision 
 Local recurrence is rare
Liposarcoma 
 Primary malignant tumor arising from fat 
 15% of all soft tissue tumors 
 Usually presents at 40-60 years of age 
 Two main types 
 Myxoid: usually low grade Stage I lesion 
 Cytogenic marker: reciprocal translocation on chromosome 12 
 Pleomorphic: usually high grade Stage II lesion
Liposarcoma 
 Can be very large at presentation due to anatomic 
location and deep-seated, slow-growing nature 
 Also typically not painful or tender 
 MRI 
 Similar to other soft tissue sarcomas, 
with multilobular configuration 
 T1) Low intensity 
 T2) Bright signal
Liposarcoma 
 Myxoid Histology 
 Sheets of lipocytes or lipoblasts interspersed in myxo-matous 
amorphous matrix with low cell to matrix ratio 
 Fine branching capillaries with arborization pattern, 
resemble a road map 
 Pleomorphic Histology 
 More cellular 
 Lacks plexiform capillary network 
 May have large, bizarre lipoblasts with abundant 
eosinophilic cytoplasm
 Treatment 
Liposarcoma 
 Preoperative adjuvant radiation therapy 
 Wide surgical excision 
 Treatment 
 Wide local excision 
 Radiotherapy may be used to control local recurrence 
and lessen the risk of metastasis 
 Prognosis 
 Five year survival: well differentiated 85-100%, 
myxoid 75-95%, round cell and pleomorphic 
20-50%, dedifferentiated 30% 
 Local recurrence 10% with adequate margins
Case 5 
T.S. - 44 year old male with six month 
history of an anterior shoulder mass
Pleomorphic Sarcoma 
 Malignant soft tissue tumor of 
mesechymal origin 
 Epidemiology 
 7000 new cases of soft tissue sarcomas 
diagnosed yearly 
 Lesions classified according to direction of 
differentiation 
 Can be caused by radiation exposure
Pleomorphic Sarcoma 
 Symptoms 
 Enlarging mass, most are large, deep and 
firm at diagnosis 
 Can be painless or painful 
 Radiographs 
 MRI is the best imaging modality
Pleomorphic Sarcoma 
 Histology 
 Dedifferentiated mesenchymal cells 
 Grade of tumors determined on histology 
 Treatment 
 Radiation therapy 
 Wide surgical excision, limb salvage if possible 
 Prognosis depends on 
 Tumor Grade 
 Size (> 5 cm) 
 Location superficial or deep to fascia
Case 6 
D.E. - 15 year old male with left knee pain after 
falling from a skateboard three weeks ago
Chondroblastoma 
 Benign tumor composed of chondroblasts 
 Epidemiology 
 Male: female is 3:1 
 10-20 years of age 
 Localization 
 Epiphysis, apophysis, or short bone 
 Extension to metaphysis and even through 
physeal plate. 
 Proximal humerus>distal femur>proximal 
tibia>proximal femur
Chondroblastoma 
 Clinical Presentation 
 Pain, usually referred to a joint 
 Moderate to long duration 
 Imaging 
 XR: Osteolytic with smooth boarders, eccentric 
 CT/MRI: Well defined boarders, fuzzy mottled 
intratumoral radio densities
 Gross 
Chondroblastoma 
 Rubbery soft with sharp limits toward the 
surrounding bone 
 Pink to gray to tan in color 
 Whitish foci or chalky granules (calcifications) 
 Histo 
 Highly cellular: Chondroblasts and Giant cells 
 Pathognomonic: calcium in fine granules 
deposited on a reticular network producing a 
“chicken-wire” pattern
 DDX 
Chondroblastoma 
 Giant Cell Tumor, Clear Cell Chondrosarcoma, 
Chondroma, Chronic Brodie’s abscess 
 Treatment 
 Stage 1, 2, and some stage 3: intralesional 
excision after frozen confirmation 
 Stage 3: wide intra or extraarticular segmental 
resection 
 Prognosis 
 Local recurrence < 10% after curettage in stage 
3 tumors, rare after wide resection
Case 7 
T.L. - 49 year old male with left elbow 
pain and swelling after a fall
Osteomyelitis 
 Bone Infection 
 Acute hematogenous, subacute focal disease, 
and chronic types 
 Epidemiology 
 Most common in infants and children 
 Declining incidence due to better dx/tx 
 Staph aureus most common in all age groups
Osteomyelitis 
 Symptoms 
 Pain, decreased ROM, swelling, erythema, 
fever, malaise, irritability 
 Radiographs 
 Delayed changes on imaging (2-4 weeks) 
 Lytic lesion with mottled appearance usually 
in metaphysis 
 Periosteal elevation/reaction
Osteomyelitis 
 Histology 
 Mixed cell population 
 Sequestrum: dead cortical bone 
 Involucrum: new cortical bone 
 Treatment 
 Biopsy/culture 
 Acute 
 IV antibiotics ± surgical debridement 
 Chronic 
 IV antibiotics with surgical debridement
Case 8 
K.M. - 13 year old female with a painful 
soft tissue mass of the left anterior knee
Epithelioid Granulomatous Inflammation 
 Chronic inflammation 
 A compact collection of cells of the mononuclear 
phagocyte system 
 Types 
 Low Turnover: Foreign body 
 High Turnover: Epithelioid, hypersensitivity 
 Treatment 
 Marginal excision
Case 9 
N.S. - 19 year old male with a chest wall mass 
discovered on an x-ray for suspected bronchitis
Calcifying Fibrous Tumor of Pleura 
 Very rare benign fibrous tumor of pleura 
 Composed of hyalinised collagen with psam-momatous 
calcification and inflammatory infiltrate 
 Rare cases reported in the literature are in patients 
less than 30 years old 
 Imaging shows circumscribed lesion with 
increased density centrally which represents 
areas of calcification 
 Treatment is typically excisional biopsy to rule 
out sarcoma
Case 10 
A.Z. - 70 year old female with left distal 
thigh pain and swelling for several weeks
B-Cell Lymphoma 
 Neoplasm of B-Lymphocytes 
 Most common Non-Hodgkin lymphoma 
 Fourth through seventh decades 
 Primary lymph node disease 
 Extranodal sites 
 More than 50% of patients will have 
extranodal involvement at diagnosis 
 Primary bone lymphomas are rare 
 (5% of extranodal disease) 
 Most common site is the thigh
Large B Cell 
Lymphoma of Soft Tissue 
 Symptoms 
 Often mild and prolonged before diagnosis 
 Radiographs 
 Lytic lesion with “moth eaten” appearance 
 Histology 
 Dense sheets of round cells 
 CD-20 positive
Large B Cell 
Lymphoma of Soft Tissue 
 Treatment 
 Combined chemotherapy and radiation to 
bony sites 
 Chemo: Cytoxan, adriamycin, vincristine, 
and prednisone 
 If remission is obtained by 6 cycles of chemo, 
cure rates approximate 60-70%
Answers (1)

Answers (1)

  • 1.
    June 2007 TumorUnknown Conference
  • 2.
    Case 1 C.G.- 21 year old male with a history of curretage and bone grafting of a distal ulnar lesion eighteen months ago
  • 3.
    Aneurysmal Bone Cyst(ABC)  Osteolytic, hyperplastic, hyperemic-hemorrhagic lesion of unknown origin  Epidemiology  80% before 20 years of age, rare after 30 years old  Most common sites: long bones LE > UE  30% secondary lesions due to pre-existing tumor  Radiographs  Eccentric, expansile, lytic lesion of metaphysis  Cortical attenuation or destruction  Rim of reactive bone  Fluid-Fluid levels on MRI are characteristic
  • 4.
    ABC  Histology  Cavernous spaces filled with blood, lacking endothelial lining  Fibroblastic cells, multinucleated giant cells and thin strands of bone
  • 5.
    ABC  Treatment  Excision, curettage, and bone grafting  Resection in case of an expendable bone.  Other modalities include adjuvant cryo, chemical cautery, injection of steroids  Radiation not recommended  Prognosis  Local recurrence rate approx 10%, increased if open physis
  • 7.
    Case 2 T.K.- 18 year old male with asymptomatic left ankle swelling for several months
  • 8.
    Aneurysmal Bone Cyst(ABC)  Osteolytic, hyperplastic, hyperemic-hemorrhagic lesion of unknown origin  Epidemiology  80% before 20 years of age, rare after 30 years old  Most common sites: long bones LE > UE  30% secondary lesions due to pre-existing tumor  Radiographs  Eccentric, expansile, lytic lesion of metaphysis  Cortical attenuation or destruction  Rim of reactive bone  Fluid-Fluid levels on MRI are characteristic
  • 9.
    ABC  Histology  Cavernous spaces filled with blood, lacking endothelial lining  Fibroblastic cells, multinucleated giant cells and thin strands of bone
  • 10.
    ABC  Treatment  Excision, curettage, and bone grafting  Resection in case of an expendable bone  Other modalities include adjuvant cryo, chemical cautery, injection of steroids  Radiation not recommended  Prognosis  Local recurrence rate approx 10%, increased if open physis
  • 12.
    Case 3 S.P.- 12 year old male with left hip pain after a minor fall
  • 13.
    Unicameral Bone Cyst  Benign fluid filled cystic lesion of bone of unknown cause  Epidemiology  5-15 years of age  Male > Female  Proximal humerus most common, followed by proximal femur
  • 14.
    Unicameral Bone Cyst  Symptoms  Asymptomatic unless fracture occurs  Radiographs  Central lytic lesion of metaphysis  Attenuation and slight expansion of cortex  Fluid filled on CT/MRI
  • 15.
    Unicameral Bone Cyst  Histology  Thin fibrous membrane lining cyst  Few macrophages, giant cells, leukocytes and slivers of bone and osteoid
  • 16.
    Unicameral Bone Cyst  Treatment  Cysts become inactive at skeletal maturity and typically resolve without surgical intervention  Multiple treatments including curettage and bone grafting, steroid injections have been tried with variable efficacy  Fractures tend to lead to resolution of cyst  Displaced fractures are the only absolute surgical indication
  • 17.
    Case 4 A.M.- 87 year old female with an asymptomatic posterior thigh soft tissue mass present for many years, the patient reports that it has increased in size over the past three months
  • 18.
    Lipoma  Benigntumor of mature fat  Classification  Superficial: (more common)  contained within subcutis  prevalence for back, neck, proximal limbs  Deep: (less common)  within muscle or intermuscular spaces  Age  Most frequent between 40-60 years old  Presentation  Slow growing asymptomatic mass
  • 19.
    Lipoma  Imaging  Resembles normal fat on all CT and MRI images  Histology  Mature fat cells (lipocytes)  Rarely small areas of hemmorrage, necrosis, calcification  Absence of lipoblasts and pleomorphic, hyperchromatic nuclei differentiate lipoma from liposarcoma  Treatment  Marginal Excision  Local recurrence is rare
  • 20.
    Liposarcoma  Primarymalignant tumor arising from fat  15% of all soft tissue tumors  Usually presents at 40-60 years of age  Two main types  Myxoid: usually low grade Stage I lesion  Cytogenic marker: reciprocal translocation on chromosome 12  Pleomorphic: usually high grade Stage II lesion
  • 21.
    Liposarcoma  Canbe very large at presentation due to anatomic location and deep-seated, slow-growing nature  Also typically not painful or tender  MRI  Similar to other soft tissue sarcomas, with multilobular configuration  T1) Low intensity  T2) Bright signal
  • 22.
    Liposarcoma  MyxoidHistology  Sheets of lipocytes or lipoblasts interspersed in myxo-matous amorphous matrix with low cell to matrix ratio  Fine branching capillaries with arborization pattern, resemble a road map  Pleomorphic Histology  More cellular  Lacks plexiform capillary network  May have large, bizarre lipoblasts with abundant eosinophilic cytoplasm
  • 23.
     Treatment Liposarcoma  Preoperative adjuvant radiation therapy  Wide surgical excision  Treatment  Wide local excision  Radiotherapy may be used to control local recurrence and lessen the risk of metastasis  Prognosis  Five year survival: well differentiated 85-100%, myxoid 75-95%, round cell and pleomorphic 20-50%, dedifferentiated 30%  Local recurrence 10% with adequate margins
  • 24.
    Case 5 T.S.- 44 year old male with six month history of an anterior shoulder mass
  • 25.
    Pleomorphic Sarcoma Malignant soft tissue tumor of mesechymal origin  Epidemiology  7000 new cases of soft tissue sarcomas diagnosed yearly  Lesions classified according to direction of differentiation  Can be caused by radiation exposure
  • 26.
    Pleomorphic Sarcoma Symptoms  Enlarging mass, most are large, deep and firm at diagnosis  Can be painless or painful  Radiographs  MRI is the best imaging modality
  • 27.
    Pleomorphic Sarcoma Histology  Dedifferentiated mesenchymal cells  Grade of tumors determined on histology  Treatment  Radiation therapy  Wide surgical excision, limb salvage if possible  Prognosis depends on  Tumor Grade  Size (> 5 cm)  Location superficial or deep to fascia
  • 28.
    Case 6 D.E.- 15 year old male with left knee pain after falling from a skateboard three weeks ago
  • 29.
    Chondroblastoma  Benigntumor composed of chondroblasts  Epidemiology  Male: female is 3:1  10-20 years of age  Localization  Epiphysis, apophysis, or short bone  Extension to metaphysis and even through physeal plate.  Proximal humerus>distal femur>proximal tibia>proximal femur
  • 30.
    Chondroblastoma  ClinicalPresentation  Pain, usually referred to a joint  Moderate to long duration  Imaging  XR: Osteolytic with smooth boarders, eccentric  CT/MRI: Well defined boarders, fuzzy mottled intratumoral radio densities
  • 31.
     Gross Chondroblastoma  Rubbery soft with sharp limits toward the surrounding bone  Pink to gray to tan in color  Whitish foci or chalky granules (calcifications)  Histo  Highly cellular: Chondroblasts and Giant cells  Pathognomonic: calcium in fine granules deposited on a reticular network producing a “chicken-wire” pattern
  • 32.
     DDX Chondroblastoma  Giant Cell Tumor, Clear Cell Chondrosarcoma, Chondroma, Chronic Brodie’s abscess  Treatment  Stage 1, 2, and some stage 3: intralesional excision after frozen confirmation  Stage 3: wide intra or extraarticular segmental resection  Prognosis  Local recurrence < 10% after curettage in stage 3 tumors, rare after wide resection
  • 34.
    Case 7 T.L.- 49 year old male with left elbow pain and swelling after a fall
  • 35.
    Osteomyelitis  BoneInfection  Acute hematogenous, subacute focal disease, and chronic types  Epidemiology  Most common in infants and children  Declining incidence due to better dx/tx  Staph aureus most common in all age groups
  • 36.
    Osteomyelitis  Symptoms  Pain, decreased ROM, swelling, erythema, fever, malaise, irritability  Radiographs  Delayed changes on imaging (2-4 weeks)  Lytic lesion with mottled appearance usually in metaphysis  Periosteal elevation/reaction
  • 37.
    Osteomyelitis  Histology  Mixed cell population  Sequestrum: dead cortical bone  Involucrum: new cortical bone  Treatment  Biopsy/culture  Acute  IV antibiotics ± surgical debridement  Chronic  IV antibiotics with surgical debridement
  • 38.
    Case 8 K.M.- 13 year old female with a painful soft tissue mass of the left anterior knee
  • 39.
    Epithelioid Granulomatous Inflammation  Chronic inflammation  A compact collection of cells of the mononuclear phagocyte system  Types  Low Turnover: Foreign body  High Turnover: Epithelioid, hypersensitivity  Treatment  Marginal excision
  • 40.
    Case 9 N.S.- 19 year old male with a chest wall mass discovered on an x-ray for suspected bronchitis
  • 41.
    Calcifying Fibrous Tumorof Pleura  Very rare benign fibrous tumor of pleura  Composed of hyalinised collagen with psam-momatous calcification and inflammatory infiltrate  Rare cases reported in the literature are in patients less than 30 years old  Imaging shows circumscribed lesion with increased density centrally which represents areas of calcification  Treatment is typically excisional biopsy to rule out sarcoma
  • 42.
    Case 10 A.Z.- 70 year old female with left distal thigh pain and swelling for several weeks
  • 43.
    B-Cell Lymphoma Neoplasm of B-Lymphocytes  Most common Non-Hodgkin lymphoma  Fourth through seventh decades  Primary lymph node disease  Extranodal sites  More than 50% of patients will have extranodal involvement at diagnosis  Primary bone lymphomas are rare  (5% of extranodal disease)  Most common site is the thigh
  • 44.
    Large B Cell Lymphoma of Soft Tissue  Symptoms  Often mild and prolonged before diagnosis  Radiographs  Lytic lesion with “moth eaten” appearance  Histology  Dense sheets of round cells  CD-20 positive
  • 45.
    Large B Cell Lymphoma of Soft Tissue  Treatment  Combined chemotherapy and radiation to bony sites  Chemo: Cytoxan, adriamycin, vincristine, and prednisone  If remission is obtained by 6 cycles of chemo, cure rates approximate 60-70%