Soft Tissue Clear Cell Sarcoma

Excerpt

Clear cell sarcoma of soft tissue (CCSST) is an exceedingly rare tumor that originates from neural crest cells and is histologically characterized by clear cells representing intracellular glycogen accumulation. These tumors share histological and immunohistochemical characteristics with malignant melanoma (MM). They commonly arise from tendinous sheaths and aponeuroses, with the majority described in the lower limbs, particularly around the ankles. Tumors arising in the upper extremities are rare, and very few cases of primary CCSST have been described as arising in the chest wall and scapular soft tissues.