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cc-by-nc-nd (c) Elsevier España, 2019
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/178626

Perivascular epitheliod cell tumors: Study of three gynecological cases

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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, FUSTÉ, Pere, SACO, Adela, ORDI I MAJÀ, Jaume, TORNÉ BLADÉ, Aureli. Perivascular epitheliod cell tumors: Study of three gynecological cases. _Medicina Clinica_. 2019. Vol. 153, núm. 2, pàgs. 83-87. [consulta: 20 de gener de 2026]. ISSN: 0025-7753. [Disponible a: https://hdl.handle.net/2445/178626]

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