The management of clear cell sarcoma

Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease-free and overall survival after resection of clear cell sarcoma in this limited study were excellent, which may be explained by relatively small tumour size in seven out of eight patients and adjuvant radiation treatment. The current treatment for clear cell sarcoma is wide local tumour excision, with adjuvant radiation therapy for resection margins of less than 1 mm.