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cc-by-nc-nd (c) Elsevier España, 2019
Please use this identifier to cite or link to this item: https://hdl.handle.net/2445/178626

Perivascular epitheliod cell tumors: Study of three gynecological cases

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Abstract

Antecedents: Perivascular epitheliod cell tumor (PEComa) is a rare mesenchymal tumor. They are rare in the field of gynecology, which makes them difficult to consider as a possible diagnostic. We aim to contribute with our experience to ease clinical practice to others gynecologists. Patients and methods: We contribute to literature with three gynecological cases; uterine, vaginal and retroperitoneal PEComas. Results: The uterine and vaginal PEComa, have required surgical treatment, and are free of disease at 9 and 5 months respectively. The retroperitoneal PEComa has recurred at 72 months of follow-up in form of retroperitoneal mass and pulmonary lymphangioleomyomatosis, continues treatment with sirolimus with good tolerance and partial response. Discussion: Given the scarcity of cases, the literature consists of case reports and mini-reviews. Some authors have categorized the PEComas based on prognostic factors, but there is no agreement regarding the follow-up and treatment. 18F-FDG-PET/CT can help characterize these lesions. The surgery is the standard. In recurrent or malignant cases, there is a lack of evidence regarding chemotherapy and radiotherapy. New therapies with inhibitory m-TOR open a hopeful therapeutic window.

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NICOLÁS, Inmaculada, et al. Perivascular epitheliod cell tumors: Study of three gynecological cases. Medicina Clinica. 2019. Vol. 153, num. 2, pags. 83-87. ISSN 0025-7753. [consulted: 17 of October of 2026]. Available at: https://hdl.handle.net/2445/178626

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