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European Journal of Gynaecological Oncology  2020, Vol. 41 Issue (4): 495-499    DOI: 10.31083/j.ejgo.2020.04.5305
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Malignant perivascular epithelioid cell tumor (PEComa) of uterus: A case report with literature review
Joseph J. Noh1, Sir-Yeon Hong1, Byeong-gie Kim1, *()
1Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 06351 Seoul, South Korea
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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.
Key words:  Perivascular epithelioid cell tumor (PEComa)      Uterus      Adjuvant chemotherapy      Vincristine      Sirolimus      Dacarbazine     
Submitted:  13 July 2019      Accepted:  09 September 2019      Published:  15 August 2020     
*Corresponding Author(s):  BYEONG-GIE KIM     E-mail:  bksong.kim@samsung.com

Cite this article: 

Joseph J. Noh, Sir-Yeon Hong, Byeong-gie Kim. Malignant perivascular epithelioid cell tumor (PEComa) of uterus: A case report with literature review. European Journal of Gynaecological Oncology, 2020, 41(4): 495-499.

URL: 

https://ejgo.imrpress.com/EN/10.31083/j.ejgo.2020.04.5305     OR     https://ejgo.imrpress.com/EN/Y2020/V41/I4/495

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