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J Pathol Transl Med. 2016 Nov;50(6):469-473. Epub 2016 Jul 25.

A Case of Malignant PEComa of the Uterus Associated with Intramural Leiomyoma and Endometrial Carcinoma.

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Department of Pathology, Korea University Guro Hospital, Korea University College of Medicine, Seoul, Korea.
Department of Obsterics and Gynecology, Korea University Guro Hospital, Korea University College of Medicine, Seoul, Korea.


Perivascular epithelioid cell tumors (PEComas) refers to a family of mesenchymal neoplasms composed of angiomyolipomas, clear cell "sugar" tumors of the lung, and lymphangioleiomyomatoses. These tumors have a distinctive and common component of perivascular epithelioid cells that show an association with blood vessel walls and immunohistochemically display myomelanocytic differentiation. The unique neoplasms have been shown to have an expanded range through a variety of case reports, including visceral, intra-abdominal, soft tissue, and bone tumors. The retroperitoneum, abdominopelvic region, and uterus have been reported to be the most common sites. Most PEComas follow a benign course. However, reports of malignant PEComas are increasing. Many papers have described uterine PEComas, but to our knowledge, there have not yet been any reports of a malignant PEComa arising concomitant with another epithelial tumor and mesenchymal tumor. We report herein the case of a 67-year-old woman who experienced a malignant uterine PEComa infiltrating a preexisting intramural leiomyoma with synchronous well differentiated endometrial carcinoma and multiple liver and lung metastases.


Immunihistochemistry; Perivascular epithelioid cell neoplasms; Uterus

Conflict of interest statement

No potential conflict of interest relevant to this article was reported.

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