Epithelioid Sarcoma

**Semantic type:** Neoplastic Process

**Definition:** An aggressive malignant neoplasm of uncertain differentiation, 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).

**Synonyms:** - ES - Epithelioid Cell Sarcoma - Epithelioid cell sarcoma - Epithelioid sarcoma - Epithelioid sarcoma