Neoplasm with perivascular epithelioid cell differentiation

Definition

A soft tissue neoplasm composed of perivascular epithelioid cells. Representative examples include angiomyolipoma, clear cell-sugar-tumor of the lung, and lymphangioleiomyomatosis.

Also known as neoplasm with perivascular epithelioid cell differentiation, PEComa, perivascular epithelioid cell neoplasm, perivascular epithelioid cell tumour, tumor with perivascular epithelioid cell differentiation, tumour with perivascular epithelioid cell differentiation — per MONDO

Also identified as

• DOID 2643 per MONDO
• MESH D054973 per MONDO
• NCIT C38150 per MONDO
• Orphanet 595133 per MONDO
• UMLS C1300127 per MONDO

Drugs indicated