Multifocal PEComa (PEComatosis) of the female genital tract associated with endometriosis, diffuse adenomyosis, and endometrial atypical hyperplasia.

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.