Synonyms: PEComa | Perivascular epithelioid tumour
A rare soft tissue tumor characterized by distinctive perivascular epitheloid cells often arranged radially around a vascular lumen as well as spindled cells in variable proportion. Melanocytic and muscle markers are typically positive. The tumors have been reported in the uterus falciform ligament and large and small intestine among others. Depending on their location they may present as a painful or painless mass or with vaginal bleeding. Tumors displaying infiltrative growth marked hypercellularity nuclear enlargement and hyperchromasia high mitotic activity atypical mitotic figures and/or coagulative necrosis should be regarded as malignant.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2026
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Perivascular epithelioid cell neoplasm?
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