Clear Cell Sarcoma (Malignant Melanoma) of Soft Parts: A Clinicopathologic Study of 52 Cases

Abstract

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 the BRAF or NRAS gene 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 of BRAF or NRAS was 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.

Clear Cell Sarcoma-like Tumor of the Gastrointestinal Tract: Clinical Outcome and Pathologic Features of a Molecularly Characterized Tertiary Center Case Series

“…Reported metastatic sites include liver, peritoneum and lymph nodes (2,8). Conventional CCS can metastasize to lung, bone and lymph nodes, and is, also, a very aggressive disease (12)(13)(14). Our case series of six patients with CCSLTGT indicates that this is a very aggressive sarcoma subtype, with 4 out of 6 patients succumbing to disease (a median survival of 8 months).…”

Clear cell sarcoma of right knee with bone marrow metastasis: A case report and review of literature.

“…Thus, imaging may be non-specific and definite diagnosis can only be made on histopathology. [7] Microscopically, CCS is composed of infiltrative epithelioid to spindle-shaped cells arranged as compact nests, fascicles and in the alveolar pattern. [2] These nests are separated by fibrous septae which are often continuous with collagen of tendons and aponeurosis.…”

Acral manifestations of soft tissue tumors

“…More aggressive care is necessary for epithelioid and clear cell sarcomas given that more than half of patients die within 10 years of these diagnoses, usually after a relentlessly progressive series of recurrences. [56][57][58] Interestingly, both tumors have a marked tendency for regional lymph node metastasis (up to nearly half of cases), an unusual feature for sarcomas, which usually disseminate hematogenously to the lungs. As a result, examination of regional lymph nodes radiographically (by sentinel lymph node biopsy or by lymphadenectomy) has been recommended.…”

“…Extended surveillance is indicated, because recurrences can appear after decades of quiescence. 55,[57][58][59][60] Acral myxoinflammatory fibroblastic sarcoma…”

“…The mass grows slowly and is sometimes present for years before presentation. 57,71 Its slow growth combined with overall circumscription and modest size (usually b4 cm) 57,72 could easily engender a deceptively benign clinical impression. …”