Soft Tissue Clear Cell Sarcoma

Excerpt

Clear cell sarcoma of soft tissue (CCSST) is an ultra-rare sarcoma originating from neural crest cells, first described by Enzinger in 1965. The neoplasm primarily affects young adults, often presenting as slowly enlarging masses in the extremities, particularly around tendons or aponeuroses. The average patient is 34, though cases range in those aged from 2 to 90 years. Due to its benign appearance, CCSST is sometimes misdiagnosed early on, but it is highly aggressive, with a 5-year survival rate of around 50%.

Diagnosis involves a combination of imaging, physical examination, and histological analysis, with confirmation through genetic testing for specific translocations. Treatment typically requires wide surgical resection with negative margins, though local recurrence and metastasis are common. Radiation and chemotherapy have shown limited effectiveness, and recent studies are exploring targeted therapies and immunotherapies.

CCSST frequently metastasizes to the lungs, lymph nodes, and bone, with other sites like the brain and abdominal organs also reported. Despite ongoing research, the prognosis remains poor, particularly for larger tumors and metastatic disease, with survival heavily dependent on early detection and tumor stage.