Korean J Obstet Gynecol.  2012 Jun;55(6):433-436. 10.5468/KJOG.2012.55.6.433.

A case of perivascular epithelioid cell tumor of the uterus

• Lee YB
• Lee YJ
• Choi YS
• Seo SK

• KMID: 1783939
• DOI: http://doi.org/10.5468/KJOG.2012.55.6.433

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.

Keyword

Perivascular epithelioid cell tumor; Uterus; HMB-45

MeSH Terms

Angiomyolipoma
Breast
Cytoplasm
Eosinophils
Epithelioid Cells
Gastrointestinal Tract
Humans
Kidney
Lung
Lymphangioleiomyomatosis
Pancreas
Perivascular Epithelioid Cell Neoplasms
Prostate
Urinary Bladder
Uterus

Figure

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  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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