Perivascular Epithelioid Cell Tumor (PEComa) of Vulva: a Rare Occurrence.

Perivascular epithelioid cell tumor (PEComa) is a tumor of mesenchymal origin, with features of perivascular epithelioid cells. The primary sites of PEComa reported are the uterus, vulva, rectum, heart, breast, urinary bladder, abdominal wall, pancreas, retroperitoneum, liver, and uterus. But what is unique is that PEComa of the gynecological tract is very rare. Uterus is the most common location of PEComa reported from female genital tract. PEComa of vulva is extremely rare. A 36-year-old woman presented with vulvar mass. Her final histopathological report came as PECOMA. Ours is the third case of PEComa vulva reported in English literature. After extensive literature search, we found only two previous cases reported of PEComa of vulva. One case was of primary PEcoma of vulva reported from Japan and other from China. Distinguishing among mesenchymal neoplasms, including PEComas, endometrial stromal sarcomas, and leiomyosarcomas, can be difficult. Careful analysis of morphologic and immunohistochemical features is of the utmost importance. Ours is the third such case of PEComa of vulva reported in English literature. © Indian Association of Surgical Oncology 2021.