. Dedifferentiated Chondrosarcoma. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 16:34. Last modified Oct 30, 2010 18:20 ver.6. Retrieved 2015-03-03, from http://www.orthopaedicsone.com/x/tQAjAQ.
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
- Very aggressive and high grade chondrosarcoma that must be aggressively txd
- Transformation to a higher grade occurs in ~11% of chondrosarcomas
- 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
- 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
- Reported in a thumb PP fxd 15 yrs previously through an enchondroma
- Ollier's disease
- 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
- Two areas may be distinctly visible on cross sectioning
- High-grade spindle cell sarcoma (not recognizable as cartilaginous in origin) next (an abrupt interface) to low-grade cartilage tissue
- 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
- Wide range of chemotherapeutic regimens have been used without proven benefit or standardization
- 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
- ~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)
- MS0812 cell line in nude mice reportedly with fibrosarcoma with chondroid components
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