Journal List > Korean J Obstet Gynecol > v.55(6) > 1088468

Lee, Lee, Choi, and Seo: A case of perivascular epithelioid cell tumor of the uterus

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.

Figures and Tables

Fig. 1
Preoperative ultrasonography. Well marginated, oval shaped hypoechoic lesion is noted on the fundus of the uterus.
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Fig. 2
Operative findings shows moderate amount of hemoperitoneum (A) and 2×2 cm sized uterine mass on uterine fundus (B).
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Fig. 3
Basic characteristic features of perivascular epithelioid cells include abundant clear to lightly eosinophilic granular cytoplasm, normochromatic ovoid nuclei (H&E, ×400) (A) with an immunoprofile of HMB-45 positivity (B).
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