A case of malignant PEComa of the uterus associated with intramural leiomyoma and endometrial carcinoma

Yoo Jin Choi, Jin-Hwa Hong, Aeree Kim, Han Kyeom Kim, Hyeyoon Chang

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)469-473
Number of pages5
JournalJournal of Pathology and Translational Medicine
Volume50
Issue number6
DOIs
Publication statusPublished - 2016

Fingerprint

Perivascular Epithelioid Cell Neoplasms
Leiomyoma
Endometrial Neoplasms
Uterus
Neoplasms
Angiomyolipoma
Epithelioid Cells
Lung
Blood Vessels
Neoplasm Metastasis
Bone and Bones

Keywords

  • Immunihistochemistry
  • Perivascular epithelioid cell neoplasms
  • Uterus

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

Cite this

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title = "A case of malignant PEComa of the uterus associated with intramural leiomyoma and endometrial carcinoma",
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.",
keywords = "Immunihistochemistry, Perivascular epithelioid cell neoplasms, Uterus",
author = "Choi, {Yoo Jin} and Jin-Hwa Hong and Aeree Kim and Kim, {Han Kyeom} and Hyeyoon Chang",
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T1 - A case of malignant PEComa of the uterus associated with intramural leiomyoma and endometrial carcinoma

AU - Choi, Yoo Jin

AU - Hong, Jin-Hwa

AU - Kim, Aeree

AU - Kim, Han Kyeom

AU - Chang, Hyeyoon

PY - 2016

Y1 - 2016

N2 - 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.

AB - 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.

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