CLEAR-CELL CHONDROSARCOMA - A REPORT OF 1 3 CASES

Purpose of the study Clear cell chondrosarcoma is a variant of bone ch ondrosarcoma. These unusual tumors are peculiar by their epiphyseal lo cation in long bones, by their cytology and by their protracted course . Clinical, radiographical and histological features are described bef ore therapeutic approach. Material and methods We report a series of t hirteen cases of clear cell chondrosarcoma identificated and treated b etween 1965 and 1995 in Cochin Hospital. Results Clear cell chondrosar coma represented 5,4 per cent of the whole chondrosarcomas registered in the same period. There was a male prevalence (8 cases). Most patien ts were in their third decade (8 cases). Clinical course was usually o f long duration ranging from three months to nine years. Roentgenograp hically, the lesion was often indistinguishable from chondroblastoma;t his tumor involved the proximal part of the femur in 8 cases, located in the epiphysis. Histologically, cells with clear cytoplasm were alwa ys present, and areas of conventional chondrosarcoma were seen in half of cases. All patients underwent surgical treatment : ''en bloc'' res ection was performed in 12 cases, after a curettage with bone grafting in 2 cases; a simple curettage with bone grafting was performed in on e case. Two recurrences occured a local recurrence after a bone grafti ng treated by resection with good result, and another recurrence with pulmonary metastasis leading to death within 8 years. Discussion Clini cal, radiographical and histological features are the same in our seri es and in the series published by Unni ef al. in 1976 and by Bjornsson et al. in 1984. Treatment consists in : ''en bloc'' resection because the lesion may recur after curettage. Rarely the tumor may metastasiz e. Prognosis is good with a five year survival rate similar to grade 1 chondrosarcoma (O'Neal and Ackerman classification). Conclusion These rare bone tumors are characterized by their good prognosis when the t reatment is an ''en bloc'' resection.