J Pathol Transl Med.  2016 Nov;50(6):469-473. 10.4132/jptm.2016.04.20.

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

• Choi YJ
• Hong JH
• Kim A
• Kim H
• Chang H

• KMID: 2361995
• DOI: http://doi.org/10.4132/jptm.2016.04.20

Abstract

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.

Keyword

Immunihistochemistry; Perivascular epithelioid cell neoplasms; Uterus

MeSH Terms

Aged
Angiomyolipoma
Blood Vessels
Endometrial Neoplasms*
Epithelioid Cells
Female
Humans
Leiomyoma*
Liver
Lung
Lymphangioleiomyomatosis
Neoplasm Metastasis
Perivascular Epithelioid Cell Neoplasms*
Uterus*

Figure

• 

  Fig. 1. Total hysterectomy showing endometriod carcinoma on anterior wall to fundus of the endometrium, and whitish leiomyoma encircling perivascular epithelioid cell tumors in the myometrium.

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  Fig. 2. (A) Endometrioid carcinoma, International Federation of Gynecology and Obstetrics (FIGO) G1. (B) Infiltrating spindle perivascular epithelioid cell tumor (PEComa) cells into surrounding leiomyoma. (C) The PEComa cells have elongated nuclei, prominent nucleoli, clear cytoplasm, cytological atypia, and necrosis.

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  Fig. 3. (A) Human melanoma black 45 staining shows diffuse cytoplasm and membrane positivity. (B) Transcription factor E3 staining showed nuclear positivity. (C) Desmin stain is negative for perivascular epithelioid cell tumor and positive for leiomyoma. Infiltrative border is identified.

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