{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T07:45:46Z","timestamp":1782546346237,"version":"3.54.5"},"reference-count":71,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T00:00:00Z","timestamp":1782432000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T00:00:00Z","timestamp":1782432000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"abstract":"<jats:p>Perivascular epithelioid cell tumours (PEComas) comprise a distinctive and biologically heterogeneous family of mesenchymal neoplasms defined by perivascular epithelioid differentiation and dual melanocytic and smooth muscle\u2010like immunophenotypes. Within the genitourinary (GU) tract, renal tumours are historically designated as angiomyolipoma (AML) and represent the most common and biologically informative manifestation of PEComa, serving as the conceptual reference model for understanding PEComa across different organ sites. Extrarenal GU PEComas, on the other hand, are rare, morphologically diverse and frequently pose significant diagnostic challenges, particularly in limited biopsy material. This review synthesizes contemporary clinicopathological, immunohistochemical and molecular data on GU PEComas, with emphasis on diagnostic pathology, risk stratification and practical diagnostic interpretation. This also emphasizes classification based on shared molecular drivers rather than organ site or legacy terminology and provides a guide for accurate diagnosis, risk assessment and interpretation of PEComas in daily pathology practice.<\/jats:p>","DOI":"10.1111\/his.70204","type":"journal-article","created":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T06:50:53Z","timestamp":1782543053000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Perivascular epithelioid cell tumours of the genitourinary tract: clinicopathological features and molecular landscape"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0537-3127","authenticated-orcid":false,"given":"Busra","family":"Yaprak Bayrak","sequence":"first","affiliation":[{"name":"Department of Pathology, School of Medicine Kocaeli University  Kocaeli Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0929-0940","authenticated-orcid":false,"given":"Mahmut","family":"Akgul","sequence":"additional","affiliation":[{"name":"Department of Pathology Brigham and Women's Hospital  Boston Massachusetts USA"},{"name":"Department of Pathology Atlas University School of Medicine  Istanbul Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6374-1303","authenticated-orcid":false,"given":"Michael","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Pathology Duke University School of Medicine  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-8803-5468","authenticated-orcid":false,"given":"Nazif Alperen","family":"Y\u0131ld\u0131r\u0131m","sequence":"additional","affiliation":[{"name":"Department of Pathology Gulhane Research and Training Hospital  Ankara Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-7233-2361","authenticated-orcid":false,"given":"Ankur R.","family":"Sangoi","sequence":"additional","affiliation":[{"name":"Department of Pathology Stanford University  Stanford California USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6049-5293","authenticated-orcid":false,"given":"Liang","family":"Cheng","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Department of Surgery (Urology) Brown University Warren Alpert Medical School, The Legorreta Cancer Center at Brown University, and Brown University Health  Providence Rhode Island USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,26]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1016\/j.eururo.2022.06.016","article-title":"The 2022 World Health Organization classification of Tumours of the urinary system and male genital organs\u2010part a: renal, penile, and testicular tumours","volume":"82","author":"Moch H","year":"2022","journal-title":"Eur. 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