Clear Cell Chondrosarcoma

A radiolucent low-grade sarcoma of the epiphysis of long bones, especially of the proximal femur, which affects all age groups; male:female ratio, 2.5:1, metastases occur to other bones and lungs
Management En bloc resection and wide margin
Prognosis 15–20% death
References in periodicals archive ?
Number of patients 44 Number of lesions treated 117 Age (range) 67 (19-91) Primary tumor histology UPS/MFH 21 Leiomyosarcoma 5 Synovial sarcoma 5 Ewings 2 Hemangiopericytoma 2 Myxofibrosarcoma 2 Spindle cell sarcoma 2 Chondrosarcoma 1 Clear cell chondrosarcoma 1 Dedifferentiated chondrosarcoma 1 Dedifferentiated liposarcoma 1 Osteosarcoma 1 Primary tumor location Extremity 36 (82%) Pelvis 4 (9%) Other 4 (9%) Primary tumor grade Low 4 (9%) Intermediate 7 (16%) High 33 (75%) Prior chemotherapy Yes 23 (52%) No 21 (48%) Prior thoracic surgery Yes 17 (39%) No 27 (61%) Extrathoracic disease Yes 16 (36%) No 28 (64%) Median number of mets treated (range) 2 (1-7) Table 2: SBRT doses and fractions.
Clear cell chondrosarcoma is an uncommon variant of chondrosarcoma that classically presents as a lytic lesion in the epiphysis of the proximal humerus or femur.
Furthermore, additional differential diagnosis should include clear cell chondrosarcoma and joint centric processes such as pigmented villonodular synovitis and geodes.
Radiologicallly clear cell chondrosarcoma may show an epiphyseal lesion with calcifications and relative circumscription.
This constellation of imaging features is helpful in differentiating an intraosseous ganglion from other radiolucent epiphyseal lesions such as chondroblastoma, giant cell tumor, simple bone cyst, clear cell chondrosarcoma (in older patients), and subchondral cyst or geode, which occurs in the setting of arthritis.
* Clear cell chondrosarcoma. Fewer than 4% of all chondrosarcomas are of the clear cell subtype.
(b) Osteosarcoma (n = 9), Ewing sarcoma (n = 2), metastatic rhabdoid tumor (n = 1), metastatic synovial sarcoma (n = 1), spindle cell sarcoma (n = 1), diffuse large B-cell lymphoma (n = 1), and clear cell chondrosarcoma (n = 1).
Differential diagnosis at this time was more limited and suggestive of GCTB based on age, location, and radiological criteria; however, aneurysmal bone cyst (ABC) and malignant tumors such as telangiectatic osteosarcoma or clear cell chondrosarcoma remained as alternative diagnoses.
1) showed an aggressive neoplastic lesion with cortical breech and large soft tissue component with invasion of lateral tibial plateau with significant soft tissue edema suggestive of GCT or clear cell chondrosarcoma. FNAC--s/o inflammatory cells and no neoplastic cells were identified.
Full browser ?