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

Gabriel Levin; Mariana Pilon Capella; Raanan Meyer; Yoav Brezinov; Walter H Gotlieb

doi:10.1007/s00404-024-07510-5

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

Journal article

Open access

Peer reviewed

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

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

Archives of gynecology and obstetrics, Vol.309(6), pp.2381-2386

06/01/2024

DOI: 10.1007/s00404-024-07510-5

PMCID: PMC11147862

PMID: 38664269

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https://doi.org/10.1007/s00404-024-07510-5View

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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.

Female

Genital Neoplasms, Female - diagnosis

Genital Neoplasms, Female - pathology

Humans

MTOR Inhibitors - therapeutic use

Neoplasm Recurrence, Local - pathology

Perivascular Epithelioid Cell Neoplasms - diagnosis

Perivascular Epithelioid Cell Neoplasms - pathology

TOR Serine-Threonine Kinases - antagonists & inhibitors

Uterine Neoplasms - diagnosis

Uterine Neoplasms - pathology

Details

Title: Subtitle: Gynecologic perivascular epithelioid cell tumors (PEComas): a review of recent evidence
Creators: Gabriel Levin - Jewish General Hospital
Mariana Pilon Capella - Instituto Brasileiro de Controle do Câncer
Raanan Meyer - Cedars-Sinai Medical Center
Yoav Brezinov - McGill University
Walter H Gotlieb - McGill University
Resource Type: Journal article
Publication Details: Archives of gynecology and obstetrics, Vol.309(6), pp.2381-2386
DOI: 10.1007/s00404-024-07510-5
PMID: 38664269
PMCID: PMC11147862
NLM abbreviation: Arch Gynecol Obstet
ISSN: 1432-0711
eISSN: 1432-0711
Language: English
Date published: 06/01/2024
Academic Unit: Internal Medicine
Record Identifier: 9984945919702771

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