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Archives of Gynecology and Obstetrics

Open Access 25-04-2024 | mTOR-Inhibitors | Review

Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence

Authors: Gabriel Levin, Mariana Pilon Capella, Raanan Meyer, Yoav Brezinov, Walter H Gotlieb

Published in: Archives of Gynecology and Obstetrics

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Abstract

Gynecologic perivascular epithelioid cell (PEC) tumors, or 'PEComas,' represent a rare and intriguing subset of tumors within the female reproductive tract. This systematic literature review aims to provide an updated understanding of gynecologic PEComas based on available literature and data. Although PEComa is rare, there are varied tumor-site presentations across gynecologic organs, with uterine PEComas being the most prevalent. There is scarce high-quality literature regarding gynecologic PEComa, and studies on malignant PEComa underscore the challenges in diagnosis. Among the diverse mutations, mTOR alterations are the most prominent. Survival analysis reveals a high rate of local recurrence and metastatic disease, which commonly affects the lungs. Treatment strategies are limited, however mTOR inhibitors have pivotal role when indicated and chemotherapy may also be used. with some cases demonstrating promising responses. The paucity of data underscores the need for multicentric studies, an international registry for PEComas, and standardized reporting in case series to enhance clinical and pathological data.
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Metadata
Title
Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence
Authors
Gabriel Levin
Mariana Pilon Capella
Raanan Meyer
Yoav Brezinov
Walter H Gotlieb
Publication date
25-04-2024
Publisher
Springer Berlin Heidelberg
Published in
Archives of Gynecology and Obstetrics
Print ISSN: 0932-0067
Electronic ISSN: 1432-0711
DOI
https://doi.org/10.1007/s00404-024-07510-5