{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T10:00:36Z","timestamp":1791453636225,"version":"4.3.3"},"reference-count":39,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2025,6,28]],"date-time":"2025-06-28T00:00:00Z","timestamp":1751068800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Gynecologic perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by the co-expression of melanocytic markers (HMB-45 and Melan-A) and smooth muscle markers (SMA, desmin, and caldesmon). The uterus is the most common organ affected, with approximately 110 cases reported worldwide, while occurrences in the cervix, vagina, ovary, and other gynecologic locations are exceptionally rare. These tumors typically present with nonspecific symptoms such as abnormal uterine bleeding and pelvic pain, often mimicking other uterine neoplasms. Histopathologically, PEComas exhibit epithelioid and spindle cell morphology with variable nuclear atypia, mitotic activity, and characteristic immunohistochemical profiles. Although most PEComas behave benignly, a subset demonstrates malignant potential, associated with larger tumor sizes, an increased mitotic index, necrosis, and vascular invasion; however, standardized diagnostic criteria remain scarce. Molecular alterations frequently involve the mTOR signaling pathway through tuberous sclerosis complex (TSC) 1 and TSC2 gene mutations, offering potential targets for therapy. Surgical resection with clear margins remains the cornerstone of treatment. For advanced or metastatic cases, mTOR inhibitors have shown promising efficacy, whereas the role of radiotherapy remains uncertain. This review aims to synthesize current knowledge regarding the epidemiology, clinical presentation, histologic features, malignant potential, and treatment of uterine PEComas, emphasizing the importance of accurate histopathological classification and molecular profiling to guide individualized therapeutic strategies.<\/jats:p>","DOI":"10.3390\/cancers17132185","type":"journal-article","created":{"date-parts":[[2025,6,30]],"date-time":"2025-06-30T06:18:36Z","timestamp":1751264316000},"page":"2185","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge"],"prefix":"10.3390","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3563-1825","authenticated-orcid":false,"given":"Reyes","family":"Oliver-Perez","sequence":"first","affiliation":[{"name":"Gynaecology Oncology Unit, Department of Obstetrics and Gynaecology, University Hospital 12 de Octubre, 28041 Madrid, Spain"},{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Ortega","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynaecology, University General Hospital of Villalba, 28400 Collado Villalba, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aranzazu","family":"Manzano","sequence":"additional","affiliation":[{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"},{"name":"Department of Medical Oncology, University Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose Manuel","family":"Estrada-Lorenzo","sequence":"additional","affiliation":[{"name":"Surest Hospital Library, 28500 Arganda del Rey, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Martinez-Lopez","sequence":"additional","affiliation":[{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"},{"name":"Department of Pathology, University Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Zabia","sequence":"additional","affiliation":[{"name":"Department of Radiology, University Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9722-5139","authenticated-orcid":false,"given":"Gregorio","family":"Lopez-Gonzalez","sequence":"additional","affiliation":[{"name":"Gynaecology Oncology Unit, Department of Obstetrics and Gynaecology, University Hospital 12 de Octubre, 28041 Madrid, Spain"},{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7166-9762","authenticated-orcid":false,"given":"Ainhoa","family":"Madariaga","sequence":"additional","affiliation":[{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"},{"name":"Department of Medical Oncology, University Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Parrilla","sequence":"additional","affiliation":[{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"},{"name":"Department of Pathology, University Hospital 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7350-4985","authenticated-orcid":false,"given":"Alvaro","family":"Tejerizo","sequence":"additional","affiliation":[{"name":"Gynaecology Oncology Unit, Department of Obstetrics and Gynaecology, University Hospital 12 de Octubre, 28041 Madrid, Spain"},{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0361-8741","authenticated-orcid":false,"given":"Blanca","family":"Gil-Iba\u00f1ez","sequence":"additional","affiliation":[{"name":"Gynaecology Oncology Unit, Department of Obstetrics and Gynaecology, University Hospital 12 de Octubre, 28041 Madrid, Spain"},{"name":"Research Institute [imas12], Complutense University of Madrid, 28040 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,6,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"176","DOI":"10.1097\/PAS.0000000000000133","article-title":"Perivascular epithelioid cell neoplasm [PEComa] of the gynecologic tract: Clinicopathologic and immunohistochemical characterization of 16 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