{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T10:51:52Z","timestamp":1781866312322,"version":"3.54.5"},"reference-count":36,"publisher":"MDPI AG","issue":"6","license":[{"start":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T00:00:00Z","timestamp":1781740800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Imaging"],"abstract":"<jats:p>Uterine perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm whose sonographic profile has not been systematically characterized. We describe an index case of malignant uterine PEComa and present a PRISMA 2020-compliant systematic review (PubMed, Scopus, Cochrane Library; search 1 March 2026) of studies reporting original ultrasound data of histologically confirmed uterine PEComa. Sonographic features were coded with MUSA\/IETA terminology; Clopper\u2013Pearson 95% confidence intervals (CI) were calculated for key proportions, and malignancy subgroups were summarized descriptively. Thirty-one cases were pooled (30 from 18 studies plus our index case; median age 41 years). The profile comprised absent acoustic shadowing in all documented cases (10\/10; 95% CI 69.2\u2013100%), moderate-to-abundant vascularisation (Color Score 3\u20134, 91.7%), variable echogenicity (heterogeneous 56.0%) and predominantly regular margins (69.6%). Preoperative misdiagnosis occurred in 100% of cases, most often as leiomyoma (41.4%). In cases with known malignancy status (n = 17), irregular margins and cystic areas appeared more often in malignant lesions, but subgroups were too small for testing. Only 4\/18 studies applied standardized terminology. Uterine PEComa shows a recurrent pattern of absent shadowing, high vascularisation and solid consistency with regular margins that may aid differential diagnosis; systematic adoption of MUSA\/IETA terminology in future reports is strongly advocated.<\/jats:p>","DOI":"10.3390\/jimaging12060268","type":"journal-article","created":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T09:18:43Z","timestamp":1781860723000},"page":"268","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ultrasound Features of Uterine Perivascular Epithelioid Cell Tumor (PEComa): A Systematic Review"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-3639-5446","authenticated-orcid":false,"given":"Laura Grazia","family":"Zomp\u00ec","sequence":"first","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari Aldo Moro, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5061-8007","authenticated-orcid":false,"given":"Giorgio Maria","family":"Baldini","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari Aldo Moro, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8972-9157","authenticated-orcid":false,"given":"Maria","family":"Bardi","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari Aldo Moro, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Salvatore","family":"Lopez","sequence":"additional","affiliation":[{"name":"Gynecologic Oncology Unit, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Calabrese","sequence":"additional","affiliation":[{"name":"Department of Radiology, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Antonietta","family":"Ramunno","sequence":"additional","affiliation":[{"name":"Department of Pathology, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-4672-5003","authenticated-orcid":false,"given":"Giuseppe","family":"Colonna","sequence":"additional","affiliation":[{"name":"Gynecologic Oncology Unit, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4006-6421","authenticated-orcid":false,"given":"Vera","family":"Loizzi","sequence":"additional","affiliation":[{"name":"Gynecologic Oncology Unit, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"},{"name":"Department of Translational Biomedicine and Neurosciences (DiBraiN), University of Bari Aldo Moro, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Arezzo","sequence":"additional","affiliation":[{"name":"Gynecologic Oncology Unit, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5745-372X","authenticated-orcid":false,"given":"Gennaro","family":"Cormio","sequence":"additional","affiliation":[{"name":"Department of Interdisciplinary Medicine (DIM), University of Bari Aldo Moro, 70124 Bari, Italy"},{"name":"Gynecologic Oncology Unit, IRCCS Istituto Tumori Giovanni Paolo II, 70124 Bari, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1370","DOI":"10.1097\/PAS.0000000000001119","article-title":"Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors","volume":"42","author":"Bennett","year":"2018","journal-title":"Am. 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