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Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of theBRAForNRASgene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median 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 (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 patients studied, and mutation ofBRAForNRASwas found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.

9000 Background: Clear-cell-sarcoma (CCS) also known as melanoma of the soft parts (MSP) is a rare cancer of adolescents and young adults arising in the deep soft tissues. Several clinical and pathological data demonstrate features typical of melanoma (the propensity for lymph node metastasis, expression of melanoma markers:HMB45, S100. Conversely, deep location in the soft tissues and recurrent t(12;22) translocation coding for the fusion protein EWS-ATF1 are rather suggestive of soft tissue sarcoma (STM). Data comparing gene expression profiles of CCS, melanomas and STM showed that CCS clustered with melanoma rather than with STM. BRAF mutations are frequently found in cutaneous melanoma (40–60%) but are not found in distinct melanoma subtypes(mucosal or uveal). Upstream of BRAF, on the same MAP kinase pathway NRAS is also frequently mutated in melanoma, with a mutually exclusive spectrum of mutations for these two genes. Our objective was to evaluate the frequency of BRAF and NRAS mutations is CCS. Met...