A case of perivascular epithelioid cell tumor of the uterus.

Lee, Yu Bin; Lee, Yoo Jin; Choi, Young Sik; Seo, Seok Kyo

doi:2012.55.6.433

Obstet Gynecol Sci > Volume 55(6); 2012 > Article

Case Report

Korean Journal of Obstetrics & Gynecology 2012;55(6):433-436.
DOI: https://doi.org/10.5468/KJOG.2012.55.6.433 Published online June 16, 2012.

A case of perivascular epithelioid cell tumor of the uterus.

Yu Bin Lee, Yoo Jin Lee, Young Sik Choi, Seok Kyo Seo

Department of Obstetrics and Gynecology, Institute of Women's Life Medical Science, Gagnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea. tudeolseo@yuhs.ac

Abstract

Perivascular epithelioid cell tumor (PEComa) is a family of rare mesenchymal tumors that include angiomyolipoma, lymphangiomyomatosis and clear cell 'sugar' tumor of the lung, with distinctive histologic and immunohistochemical features. They can originate in any visceral organ such as kidney, lung, breast, urinary bladder, pancreas, prostate and gastrointestinal tract, and there are few reports of uterine PEComa. Histopathologically, the tumor consisted of nests or sheets of epithelioid cells with eosinophilic cytoplasm and positivity for melanocytic markers (especially HMB-45) plays an important role in the diagnosis of this tumor. Due to their rarity and varied sites and presentation, the biological behavior of PEComa has not yet been documented. We report a case of uterine PEComa confirmed by immunohistochemical staining and the histopathologic findings with a brief review of the literatures.

Key Words: Perivascular epithelioid cell tumor, Uterus, HMB-45