Epithelioid sarcoma (ES) is a rare, malignant soft tissue tumor. It accounts for less than 1% of all soft tissue sarcomas. ES can mimic other dermatological entities, confounding the diagnosis.

ES presents usually as an indolent, hard, tender nodule on the distal extremities of a young person (typically less than 40 years). Men are preferentially affected. Lesions on the head and neck have been reported. Aberrant mesenchymal tissue differentiation may contribute to the pathogenesis of ES. A history of trauma may precede the eruption of this lesion, although a causal relationship has not been established. The lesions often ulcerate.

ES often has an unpredictable clinical course. ES often has a tendency for recurrence and aggressive growth. Regional lymph node involvement and distant metastases to the lung, bones, brain, and other organs, and spread along connective tissue planes and neurovascular bundles, have been reported. A delay in diagnosis is not uncommon.

In a proximal / axial variant, with an adverse prognosis due to higher recurrence, earlier metastasis has been described. Ulcerating, angiomatoid / angiosarcoma-like, and fibrous histiocytoma-like / fibroma-like variants have also been reported.