Naveed Shah, Zahoor Sheikh, Batoo Azhar Jan, Haji Altaf Gowhar, Mir Abdul Wahid, Qazi Ifrah Ahmad
Department of Surgical Oncology, SKIMS Kashmir, Green Avenue colony sector 1st house 10, Peerbagh, Srinagar, Jammu Kashmir 190014 India.
Indian J Surg Oncol. 2022 Jun;13(2):242-244. doi: 10.1007/s13193-021-01437-y. Epub 2021 Sep 8.
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.
血管周上皮样细胞肿瘤（PEComa）是一种间叶源性肿瘤，具有血管周上皮样细胞的特征。报道的PEComa的原发部位有子宫、外阴、直肠、心脏、乳腺、膀胱、腹壁、胰腺、腹膜后、肝脏和子宫。但独特的是，生殖道的PEComa非常罕见。子宫是女性生殖道报道中PEComa最常见的部位。外阴的PEComa极为罕见。一名36岁女性出现外阴肿物。她最终的组织病理学报告显示为PECOMA。我们的病例是英文文献中报道的第三例外阴PEComa。经过广泛的文献检索，我们发现之前仅报道过两例外阴PEComa。一例是来自日本报道的原发性外阴PEcoma，另一例来自中国。鉴别间叶性肿瘤，包括PEComas、子宫内膜间质肉瘤和平滑肌肉瘤，可能很困难。仔细分析形态学和免疫组化特征至关重要。我们的病例是英文文献中报道的第三例此类外阴PEComa。
