RT  - European Journal of Gynaecological Oncology
ID  - 10.31083/j.ejgo.2020.04.5305
T1  - Malignant perivascular epithelioid cell tumor (PEComa) of uterus: A case report with literature review
A1  - Joseph J. Noh
A1  - Sir-Yeon Hong
A1  - Byeong-gie Kim
K1  - Perivascular epithelioid cell tumor (PEComa); Uterus; Adjuvant chemotherapy; Vincristine; Sirolimus; Dacarbazine
YR  - 2020
SP  - 495
AB  - <p>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.</p>