Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined\r\ndue to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52\r\npatients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied\r\nin 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women,\r\nwith a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors).\r\nMedian initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases\r\n(26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31\r\npatients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumorassociated\r\nparameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized\r\ndisease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT)\r\n(10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59%\r\nand 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the\r\noptimal treatment for this aggressive chemoresistant tumor.
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