PEComa

**Semantic type:** Neoplastic Process

**Definition:** A soft tissue mesenchymal tumor with perivascular epithelioid cell differentiation. Representative examples include angiomyolipoma, clear cell-sugar-tumor of the lung, and lymphangioleiomyomatosis.

**Synonyms:** - Neoplasm with Perivascular Epithelioid Cell Differentiation - Tumor with Perivascular Epithelioid Cell Differentiation