{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,22]],"date-time":"2026-08-22T12:53:08Z","timestamp":1787403188461,"version":"build-2736575974"},"reference-count":15,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T00:00:00Z","timestamp":1782950400000},"content-version":"vor","delay-in-days":1,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"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":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Biphenotypic sinonasal sarcoma (BSNS) is a rare, low\u2010grade spindle cell sarcoma of the sinonasal tract. It is characterized by dual neural and myogenic differentiation and classically shows fusions involving\n                    <jats:italic>PAX3<\/jats:italic>\n                    .\n                    <jats:italic>MAML3<\/jats:italic>\n                    , a co\u2010activator in the Notch signaling pathway, is the most common fusion partner of\n                    <jats:italic>PAX3<\/jats:italic>\n                    . Here, we describe the first reported case, to our knowledge, of BSNS harboring a novel\n                    <jats:italic>PAX3::MAML2<\/jats:italic>\n                    fusion in a polypoid lesion arising from the left ethmoid of a 61\u2010year\u2010old man. Microscopically, the tumor demonstrated bland spindle cell morphology. The cells were arranged in small fascicles and a vaguely whorled pattern without mitotic activity or cellular atypia. Immunohistochemistry displayed characteristic co\u2010expression of S100 and smooth muscle actin as well as patchy nuclear positivity for beta\u2010catenin. SOX10, AE1\/AE3, EMA, and CD34 were negative. Ki\u201067 was expressed in less than 1% of tumor cells, consistent with a low\u2010grade lesion. The RNA sequencing identified a novel\n                    <jats:italic>PAX3::MAML2<\/jats:italic>\n                    fusion transcript.\n                    <jats:italic>MAML2<\/jats:italic>\n                    fusion is commonly associated with other head and neck neoplasms, including mucoepidermoid carcinoma and\n                    <jats:italic>NR1D1<\/jats:italic>\n                    \u2010rearranged tumors. These findings highlight the value of comprehensive fusion testing in BSNS and emphasize the evolving fusion landscape in this entity and related tumors.\n                  <\/jats:p>","DOI":"10.1002\/gcc.70153","type":"journal-article","created":{"date-parts":[[2026,7,3]],"date-time":"2026-07-03T04:36:56Z","timestamp":1783053416000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Biphenotypic Sinonasal Sarcoma With a Novel\n                    <i>PAX3::MAML2<\/i>\n                    Fusion"],"prefix":"10.1002","volume":"65","author":[{"given":"Mayu","family":"Uemura","sequence":"first","affiliation":[{"name":"Department of Pathology Monash Health  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Fong Na","family":"Goh","sequence":"additional","affiliation":[{"name":"Department of Pathology Monash Health  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Pathology Monash Health  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gary","family":"Quagliotto","sequence":"additional","affiliation":[{"name":"Sullivan Nicolaides Pathology  Bowen Hills, QLD Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7755-8745","authenticated-orcid":false,"given":"Pranav","family":"Dorwal","sequence":"additional","affiliation":[{"name":"Department of Pathology Monash Health  Clayton Victoria Australia"},{"name":"School of Clinical Sciences Monash University  Clayton Victoria Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,2]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2018\u20100207\u2010RA"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182426886"},{"key":"e_1_2_10_4_1","unstructured":"WHO Classification of Tumours Editorial Board.Head and Neck Tumours. Lyon (France): International Agency for Research on Cancer; 2023. (WHO Classification of Tumours Series 5th ed.; Vol. 9) https:\/\/publications.iarc.who.int\/629."},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAP.0000000000000462"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000492"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ehpc.2021.200491"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428\u2010018\u20102426\u2010x"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13045"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2026.106076"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12105\u2010023\u201001566\u20106"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428\u2010023\u201003731\u20102"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.23032"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2989"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000001092"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1177\/10668969231204961"}],"container-title":["Genes, Chromosomes and Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/gcc.70153","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/gcc.70153","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/gcc.70153","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T10:50:50Z","timestamp":1785322250000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/gcc.70153"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":15,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1002\/gcc.70153"],"URL":"https:\/\/doi.org\/10.1002\/gcc.70153","archive":["Portico"],"relation":{},"ISSN":["1045-2257","1098-2264"],"issn-type":[{"value":"1045-2257","type":"print"},{"value":"1098-2264","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"2026-06-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-29","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-07-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70153"}}