Multifocal PEComa (PEComatosis) of the Female Genital Tract Associated With Endometriosis, Diffuse Adenomyosis, and Endometrial Atypical Hyperplasia

Elisabetta Froio, Simonetta Piana, Alberto Cavazza, Riccardo Valli, M Abrate, Martino Abrate, Giorgio Gardini
International journal of surgical pathology · 2008 · vol. 16(4) , pp. 443–446 · doi:10.1177/1066896908316067 · PMID:18499690 · W2090965285
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⚙ AI-generated summary by claude@2026-06, 2026-06-09 ⓘ

This paper reports a case of multifocal PEComatosis in a woman with tuberous sclerosis, endometriosis, adenomyosis, and atypical endometrial hyperplasia, presenting as multiple nodules in the pelvis composed of perivascular epithelioid cells.

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Abstract

The authors describe a case of multifocal perivascular epithelioid cell tumor (PEComa) arising in the pelvis of a 39-year-old woman affected by tuberous sclerosis. The tumor presented in the form of multiple fascicular, focally cystic nodules involving the uterine corpus, both ovaries, and the omentum. Microscopically, the nodules were composed of foci of adenomyosis and endometriosis (with focal atypical complex hyperplasia) associated with a stromal spindle cell population immunoreactive for HMB-45, smooth muscle actin, and estrogen and progesterone receptors. We interpret these foci as the result of a widespread proliferation of perivascular epithelioid cells (PEC). Because of the diffuse quality of the process, the designation of PEComatosis seems warranted.

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Condition tags

endometriosisadenomyosis

MeSH descriptors

Carcinoma Endometrial Hyperplasia Endometriosis Epithelioid Cells Genital Neoplasms, Female Adult Carcinoma Carcinoma Carcinoma Endometrial Hyperplasia Endometrial Hyperplasia Endometrial Hyperplasia Endometriosis Endometriosis Endometriosis Epithelioid Cells Female Genital Neoplasms, Female Genital Neoplasms, Female Genital Neoplasms, Female

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References (22)

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