• Inmaculada Nicolás, Pere Fusté, Adela Saco, Jaume Ordi, and Aureli Torné.
• Institute Clinic of Gynecology, Obstetrics, and Neonatology, Hospital Clínic - Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain; Institut de Salut Global de Barcelona (ISGlobal), Barcelona, Spain. Electronic address: innipe@hotmail.com.
• Med Clin (Barc). 2019 Jul 19; 153 (2): 83-87.

AntecedentsPerivascular 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 MethodsWe contribute to literature with three gynecological cases; uterine, vaginal and retroperitoneal PEComas.ResultsThe 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.DiscussionGiven 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.Copyright © 2019 Elsevier España, S.L.U. All rights reserved.

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