Systematic Analysis of Perivascular Epithelioid Cell Neoplasms in the Female Reproductive Tract: A Comprehensive Review

Abstract

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.

Keywords::

• female genital tract
• imaging
• immunohistochemical detection
• perivascular epithelioid cell neoplasm
• treatment

Author contributions

Q Cui: conceptualization, literature review, data curation, formal analysis, investigation, methodology, writing original draft; C Li: literature review, data curation, formal analysis, investigation, methodology; T Huang: literature review, data curation, formal analysis, investigation, methodology, writing original draft; J Huang: data curation, investigation, methodology; M Chen: conceptualization, literature review, supervision; S Yao: writing guidance; M He: writing guidance.

Financial disclosure

The authors have no financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending or royalties.

Competing interests disclosure

The authors have no competing interests or relevant affiliations with any organization or entity with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, stock ownership or options and expert testimony.

Writing disclosure

No writing assistance was utilized in the production of this manuscript.