Neoplasm with perivascular epithelioid cell differentiation

rare

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

Identifiers: MONDO:0006359 Orphanet:595133
Part of: Soft tissue neoplasm
Also known as: PEComa · perivascular epithelioid cell neoplasm · perivascular epithelioid cell tumour · tumor with perivascular epithelioid cell differentiation · tumour with perivascular epithelioid cell differentiation

Subtypes

All diseases Rare only

Disease	ID	Subtypes
Angiomyolipoma	MONDO:0002603	4
Benign PEComa	MONDO:0020581	3
Lung PEComa	MONDO:0020588	2
Lymphangioleiomyomatosis	MONDO:0011705	1
Lymphangiomyoma	MONDO:0006842
Uterine corpus perivascular epithelioid cell tumor	MONDO:0004221

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