Malignant PEComa

**Semantic type:** Neoplastic Process

**Definition:** A usually large and aggressive tumor with perivascular epithelioid cell differentiation characterized by the presence of marked nuclear atypia, pleomorphism, increased mitotic activity, necrosis, and infiltrative margins. The most common metastatic sites are liver, lungs, lymph nodes, and bone.

**Synonyms:** - PEComa, malignant - Perivascular epithelioid cell tumor, malignant