Clear cell sarcomas of the tendon sheath. An experience of 22 cases seen over 16 years.

ABSTRACT

Twenty-two histologically confirmed cases of Clear Cell Sarcomas (C.C.S.) seen over a sixteen year period, are retrospectively analysed from a clinico-pathological view-point. The tumours occurred in young adults, average 36 years. Males predominated, and the lower extremity particularly the thigh and foot were the most commonly involved. Grossly, a connection with aponeurosis and tendon sheath has not been recorded consistently. Microscopically the tumour was distinctive, with their short fascicles and nests of polygonal to fusiform cells. The "clear" cytoplasm stressed in it's very name was not the rule. Melanin was demonstrated in 7 of the 22 cases, i.e. 32%, and these patients did not behave any differently from those where pigment was not seen. Immunocytochemistry for S-100 protein was done in 14 cases and was positive in 13 cases. Follow-up information revealed that 11 patients were dead of advanced disease in an average period of 24 months. Two patients were alive with disease for an average period of 3 years 7 months. Only 4 patients had no evidence of disease for periods ranging from 6 months to 26 months. Metastasis to nodes was a very frequent event, but lung and bony metastasis has also been documented.