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WHO Classification of Tumours
Epithelioid sarcoma
Connective, subcutaneous and other soft tissues


Definition

An aggressive malignant neoplasm of uncertain lineage, characterized by the presence of epithelioid cells forming nodular patterns. The nodules often undergo central necrosis, resulting in a pseudogranulomatous growth pattern. It usually occurs in young adults. The most common sites of involvement are the extremities (distal-type epithelioid sarcoma), and less frequently the pelvis, perineum, and genital organs (proximal-type epithelioid sarcoma).