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Systematic analysis of perivascular epithelioid cell neoplasms in the female reproductive tract: a comprehensive review

    Qiulin Cui

    Department of Gynecology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    ,
    Cheng Li

    Department of Gynecology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    ,
    Tongyi Huang

    Department of Clinical Medical Ultrasonics, Division of Interventional Ultrasound, Institute of Diagnostic & Interventional Ultrasound, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    ,
    Jiaming Huang

    Department of Gynecology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    &
    Ming Chen

    *Author for correspondence:

    E-mail Address: chenm229@mail.sysu.edu.cn

    Department of Gynecology, First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China

    Published Online:https://doi.org/10.2217/fon-2023-0778

    Perivascular epithelioid cell neoplasms (PEComas) are rare mesenchymal lesions, with gynecological PEComas accounting for just over a quarter of cases. Limited reports exist on gynecological PEComa, primarily treated with surgery; adjuvant therapy is considered in high-risk cases. This systematic review aims to summarize the origin and clinical, pathological and molecular characteristics of uterine PEComa, focusing on treatment options for gynecological PEComa. A comprehensive PubMed review of gynecological PEComa reports was conducted. A detailed examination of the literature ensured a thorough understanding. Gynecological PEComa diagnosis relies on histology and immunology. Despite therapy controversies, surgery remains the mainstay. Adjuvant therapy efficacy in high-risk cases is uncertain. mTOR inhibitors are the first line; alternative treatments, including angiogenesis and aromatase inhibitors, should be considered.

    Papers of special note have been highlighted as: • of interest

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