{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,29]],"date-time":"2025-08-29T06:40:06Z","timestamp":1756449606375,"version":"3.44.0"},"reference-count":39,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,8,18]],"date-time":"2025-08-18T00:00:00Z","timestamp":1755475200000},"content-version":"vor","delay-in-days":17,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Perivascular epithelioid cell tumours (PEComas) constitute a unique group of neoplasms with a distinctive myomelanocytic immunohistochemical phenotype and uncommonly occur in the head and neck region. We herein report the clinicopathologic features of a case of a sinonasal PEComa occurring in an 18\u2010year\u2010old male with a novel <jats:italic>TRAF3::TFE3<\/jats:italic> fusion.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Presentation<\/jats:title><jats:p>The patient presented with an infiltrative nasal cavity tumour. Histologic examination showed a tumour composed of sheets and nests of epithelioid cells with clear to eosinophilic granular cytoplasm, round to oval nuclei, inconspicuous nucleoli and no mitotic activity. The tumour cells featured strong diffuse expression of smooth muscle actin and nuclear TFE3, with patchy expression of HMB45. Neoplastic cells showed no immune\u2010reactivity for Melan A. Molecular genetic analysis revealed a novel <jats:italic>TRAF3(ex8)::TFE3(ex6)<\/jats:italic> fusion.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This report contributes to the expanding molecular spectrum of <jats:italic>TFE3<\/jats:italic>\u2010altered PEComas of the head and neck region and discusses the differential diagnoses of these tumours. We further describe the evolving understanding of <jats:italic>TFE3<\/jats:italic>\u2010altered PEComas.<\/jats:p><\/jats:sec>","DOI":"10.1002\/gcc.70075","type":"journal-article","created":{"date-parts":[[2025,8,18]],"date-time":"2025-08-18T13:54:23Z","timestamp":1755525263000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<i>TFE3<\/i>\u2010Altered Perivascular Epithelioid Cell Tumour (<scp>PEComa<\/scp>) of the Nasal Cavity With a Novel <i>TRAF3::TFE3<\/i> Fusion\u2014A Report of a Case Expanding the Molecular Genetic Spectrum and a Brief Literature Review on <scp>PEComas<\/scp> of the Head and Neck Region"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-5820-1706","authenticated-orcid":false,"given":"Zhengyu Samuel","family":"Chua","sequence":"first","affiliation":[{"name":"Department of Pathology National University Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Michal","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine in Pilsen Charles University  Prague Czech Republic"},{"name":"Biopticka Laboratory, Ltd  Pilsen Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fredrik","family":"Petersson","sequence":"additional","affiliation":[{"name":"Department of Pathology National University Hospital  Singapore"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,8,18]]},"reference":[{"key":"e_1_2_8_2_1","unstructured":"L. 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