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Dedifferentiated Chondrosarcoma

DEFINITION AND PATHOGENESIS

  • A conventional chondrosarcoma juxtaposed next to a high-grade (non-cartilaginous) sarcoma
  • Two groups of precursor lesions have been identified
  • Low-grade (to high-grade) malignant chondrosarcoma
  • Moderate- to high-grade malignant chondrosarcoma, osteosarcoma, MFH, fibrosarcoma, or unspecified and anaplastic spindle-cell sarcoma

IMPORTANCE

  • Very aggressive and high grade chondrosarcoma that must be aggressively txd
  • Transformation to a higher grade occurs in ~11% of chondrosarcomas

CLINICAL FEATURES

  • 6th decade disease on the average (wide age range)
    • Mean age 59
  • M (53%) > F
  • Pain, swelling
  • 10-29% present with pathologic fx (rare for conventional chondrosarcoma)
  • Most in femur or pelvis
  • Unusual location
    • Maxilla
      • Facial swelling, epistaxis and proptosis reported
    • Primary lung location reported in a pt with systemic sclerosis
      • Sxs from a large pleural effusion were present

RADIOLOGIC FEATURES

  • Second area with aggressive characteristics
  • Soft tissue mass with penetrated/destroyed cortex (72%) adjacent to a less aggressive area
    • Type 1: resembles conventional central chondrosarcoma with cortical penetration, soft tissue mass, pathological fx, angiographic contrast uptake, extraosseous ossification, rapid progression
    • Type 2: resembles conventional central chondrosarcoma with an aggressive component
    • Type 3: no resemblance radiographically to conventional central chondrosarcoma
  • Nearly 2/3 may be associated with a secondary chondrosarcoma
    • Enchondroma
      • Reported in a thumb PP fxd 15 yrs previously through an enchondroma
    • Ollier's disease
    • Osteochondromatosis
  • Most often > 7cm
  • ± pathological fx
  • Diaphyseal or metaphyseal
  • Typically in the pelvis, femur, humerus
  • Has been reported arising in a solitary osteochondroma 
    • dedifferentiated component can be leiomyosarcoma, osteosarcoma, MFH, and fibrosarcoma
  • 3 categories of plain radiographic appearance
    • Type I: cortex not deformed, no periosteal reaction, calcific matrix, and an indolent osteolytic reion with indistinct margins
    • Type II: cortical expansion and/or with osteolytic areas
    • Type III: cortex destroyed with osteolytic regions predominate

GROSS PATHOLOGY

  • Two areas may be distinctly visible on cross sectioning

HISTOLOGIC FEATURES

  • High-grade spindle cell sarcoma (not recognizable as cartilaginous in origin) next (an abrupt interface) to low-grade cartilage tissue
  • Osteosarcoma
  • Fibrosarcoma
  • Precursor lesion may be seen
  • Dedifferentiated tumor in LR of previously low-grade chondrosarcoma (review previous pathology)

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Sarcomatous transformation of a bone infarct
  • Conventional chondrosarcoma
  • GCT due to lucency when in metaepiphyseal location

DISEASE COURSE AND TREATMENT

  • Dx with FNA reported in a pelvic location
    • (Presence of both low and high grade components documented)
  • Wide resection and chemotx
    • Wide range of chemotherapeutic regimens have been used without proven benefit or standardization
      • Doxorubicin and cisplatin
      • Doxorubicin and ifosfamide
  • XRT may be used preop in some of the larger lesions where margins may be close; the theory is that the tumor cells will not be able to seed the wound if a positive margin is obtained
  • Lack of response to chemotx may be related to high likelihood of P-glycoprotein expression
  • Pulmonary, skeletal, and visceral metastases occur with high risk
    • Skin metastasis has been reported dxd with FNA
    • 21% with metastases at the time of dx
      • Median survival of 5 mos
        • 10% 2-yr-survival
  • ~7-24% 5-yr survivals have been reported (median survival < 9 months)
    • With no metastases at presentation, overall survival 28% at 10 yrs reported
  • Best prognosis in radiologic type 1 lesions
  • Grade of dedifferentiated sarcoma the most important predictor of biological behavior (rather than type of tumor differentiation--osteoblastic or fibroblastic)

SPECIAL CONSIDERATION


  • Animal Model


    • MS0812 cell line in nude mice reportedly with fibrosarcoma with chondroid components

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