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We report a malignant uterine PEComa in a 44-year-old woman presenting with abnormal uterine bleeding and pelvic pain. Preoperative imaging suggested a fibroid, but definitive diagnosis followed hysterectomy: in fact, histopathology showed epithelioid cells arranged around a rich vasculature with high-grade cytologic atypia, brisk mitotic activity, and necrosis, all features consistent with malignant behavior. Subsequent immunophenotyping demonstrated co-expression of melanocytic and smooth-muscle markers. The disease was organ-confined; no adjuvant therapy was administered, and the patient remains disease-free at 24 months under structured surveillance. To contextualize this case, we conducted a narrative review of the literature through October 2025, synthesizing epidemiology, pathogenesis, diagnosis, histology, treatment, and outcomes of uterine PEComas. Evidence supports complete surgical excision as the cornerstone of management for localized disease. For advanced or recurrent tumors, inhibitors of the mammalian target of rapamycin (mTOR) represent a rational option given frequent dysregulation of this pathway. Overall, malignant uterine PEComa requires multidisciplinary evaluation, pathology-driven diagnosis, and long-term follow-up; accumulating case-based evidence is refining risk stratification and informing the selective use of targeted therapies.<\/jats:p>","DOI":"10.1007\/s00432-026-06487-9","type":"journal-article","created":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T07:28:21Z","timestamp":1779262101000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Malignant uterine PEComa: a narrative literature review and challenging case report"],"prefix":"10.1007","volume":"152","author":[{"given":"Mauro Francesco Pio","family":"Maiorano","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joana","family":"Sorino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mario","family":"Della Mura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doriana","family":"Di Nanni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerardo","family":"Cazzato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Rizzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stella","family":"D\u2019Oronzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Cerbone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ettore","family":"Cicinelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Marinaccio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,5,20]]},"reference":[{"key":"6487_CR35","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1186\/1746-1596-2-45","volume":"2","author":"HB Armah","year":"2007","unstructured":"Armah HB, Parwani AV (2007) Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. 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