IMR Press / EJGO / Volume 41 / Issue 4 / DOI: 10.31083/j.ejgo.2020.04.5305
Open Access Review
Malignant perivascular epithelioid cell tumor (PEComa) of uterus: A case report with literature review
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1 Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 06351 Seoul, South Korea
Eur. J. Gynaecol. Oncol. 2020 , 41(4), 495–499; https://doi.org/10.31083/j.ejgo.2020.04.5305
Submitted: 13 July 2019 | Accepted: 9 September 2019 | Published: 15 August 2020
Abstract

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm involving various organs. Approximately 70 cases of gynecologic PEComas have been reported in the literature. Due to the paucity of cases, there is no definite treatment regimen established. We describe disease progression in a case of uterine PEComa with pulmonary metastasis in a 37-year-old woman who had been treated with surgery and various regimens of adjuvant treatment including vincristine, ifosfamide, cisplatin, sirolimus, doxorubicin, olaratumab, pazopanib and dacarbazine. Adequate management and prognosis still seem inconclusive and further case reports with randomized trials are needed to delineate the nature of this neoplasm.

Keywords
Perivascular epithelioid cell tumor (PEComa)
Uterus
Adjuvant chemotherapy
Vincristine
Sirolimus
Dacarbazine
Figures
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