Clear cell sarcoma. A clinicopathologic study of 27 cases

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Abstract
Clear cell sarcoma is an indolent tumor of uncertain histogenesis. Twenty-seven patients ranging in age from 9 to 57 years (average, 28.5 years) were followed an average of 7.0 years. Females outnumbered males by 2 to 1. Distal extremity locations predominated. At presentation, the tumor was localized in 21 patients, regionally metastatic in 5, and disseminated in 1. Surgery was the primary therapy for 26 patients. Adjuvant treatment was nonstandardized, and its effectiveness is undetermined. Local recurrence developed in 10 patients, regional metastases in 9, and widespread dissemination in 12. Twelve patients died of the disease from 7 months to 10 years after diagnosis. Only 11 patients remained free of disease. It would appear that wide excision, or perhaps even radical excision or amputation, is the surgical treatment of choice.