Palmar localization of an epithelioid sarcoma minicking palmar fibromatosis

A case of epithelioid sarcoma localized in the palm of the left hand in a 3 2-year-old woman is reported. The tumor evolved for many years, clinically and microscopically simulating palmar fibromatosis. Epithelioid sarcoma is an uncommon malignant tumor, often misdiagnosed by s urgeons and pathologists. It occurs in young patients and is often localize d at the upper distal extremity. Microscopically epithelioid sarcoma shows nodules manifesting fibrous hyali ne cores with central necrosis. It contains epithelioid and spindle cells i mmunoreactive to keratin, epithelial membrane antigen and vimentin. Recurre nces, lymph node metastases and lung metastases are frequent. Surgical lite rature tends to recommend wide "en bloc" excision or amputation, combined o r not with adjuvant radiation therapy.