{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T14:42:30Z","timestamp":1791816150426,"version":"4.3.4"},"reference-count":39,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T00:00:00Z","timestamp":1698019200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Genes Chromosomes &amp;amp; Cancer"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Smooth muscle tumors are the most common mesenchymal tumors of the female genital tract, including the vulva. Since vulvar smooth muscle tumors are rare, our understanding of them compared to their uterine counterparts continues to evolve. Herein, we present two cases of morphologically distinct myxoid epithelioid smooth muscle tumors of the vulva with novel <jats:italic>MEF2D<\/jats:italic>::<jats:italic>NCOA2<\/jats:italic> gene fusion. The tumors involved 24 and 37\u2010year\u2010old women. Both tumors presented as palpable vulvar masses that were circumscribed, measuring 2.8 and 5.1\u2009cm in greatest dimension. Histologically, they were composed of epithelioid to spindle\u2010shaped cells with minimal cytologic atypia and prominent myxoid matrix. Rare mitotic figures were present (1\u20133 mitotic figures per 10 high\u2010power field (HPF)), and no areas of tumor necrosis were identified. By immunohistochemistry, the neoplastic cells strongly expressed smooth muscle actin, calponin, and desmin, confirming smooth muscle origin. Next\u2010generation sequencing identified identical <jats:italic>MEF2D<\/jats:italic>::<jats:italic>NCOA2<\/jats:italic> gene fusions. These two cases demonstrate that at least a subset of myxoid epithelioid smooth muscle tumors of the vulva represent a distinct entity characterized by a novel <jats:italic>MEF2D<\/jats:italic>::<jats:italic>NCOA2<\/jats:italic> gene fusion. Importantly, recognition of the distinct morphologic and genetic features of these tumors is key to understanding the biological potential of these rare tumors.<\/jats:p>","DOI":"10.1002\/gcc.23209","type":"journal-article","created":{"date-parts":[[2023,10,23]],"date-time":"2023-10-23T15:23:56Z","timestamp":1698074636000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Myxoid epithelioid smooth muscle tumor of the vulva: A distinct entity with <scp><i>MEF2D<\/i><\/scp>::<scp><i>NCOA2<\/i><\/scp> gene fusion"],"prefix":"10.1002","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2989-3282","authenticated-orcid":false,"given":"Laura M.","family":"Warmke","sequence":"first","affiliation":[{"name":"Department of Pathology Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amin","family":"Mustafa","sequence":"additional","affiliation":[{"name":"Department of Pathology Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying S.","family":"Zou","sequence":"additional","affiliation":[{"name":"Department of Pathology The Johns Hopkins Hospital  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2725-1518","authenticated-orcid":false,"given":"Jessica L.","family":"Davis","sequence":"additional","affiliation":[{"name":"Department of Pathology Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas M.","family":"Ulbright","sequence":"additional","affiliation":[{"name":"Department of Pathology Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila E.","family":"Segura","sequence":"additional","affiliation":[{"name":"Department of Pathology Indiana University School of Medicine  Indianapolis Indiana USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,23]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.path.2019.02.004"},{"key":"e_1_2_6_3_1","unstructured":"KurmanRJ HerringtonCS YoungRH et al.World Health Organization classification of tumours of female reproductive organs. 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