Perivascular epithelioid cell tumor of the uterus:report of three cases

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Perivascular epithelioid cell rumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The family of PEComa includes angio-myolipoma (AML), clear cell "sugar" tumors of the lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumor of the falciform/ligamentum teres (CCMT), and clear cell tumors of the pancreas,rectum, peritoneum, uterine, vagina, thigh, and heart.They are very rare besides AML, CCST, and LAM.1 To our knowledge, only 84 cases of PEComa have been reported, 30 of them occurred in the uterus.2,3 Here we report 3 cases of uterine PEComa and discuss its pathological features and differential diagnosis.
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