Contributors: Diva R. Salomão and R. Jean Campbell
Clear cell chondrosarcoma is an extremely rare variant of the chondrosarcoma characterized histopathologically by relatively large cells with clear cytoplasm, osteoclastic giant cells and areas of ossification and calcification. The clear cells have round to oval nuclei and may contain nucleoli. The clear cell chondrosarcoma has a relatively favorable prognosis and have a five year survival of 85%. It affects males more often than females (2.5:1). The tumor occurs in the epiphysis of long bones. The most common site is the head of the femur. When small a clear cell chondrosarcoma may be extremely difficult to distinguish from a chondroblastoma radiologically. The tumor has multiple foci of calcification and ossification within a myxoid matrix of cartilaginous tissue.