{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T06:44:49Z","timestamp":1791873889198,"version":"4.3.4"},"reference-count":21,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,4,3]],"date-time":"2019-04-03T00:00:00Z","timestamp":1554249600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Department of Pathology and Laboratory Medicine Fund for Molecular Pathology, QEII Foundation"},{"name":"Nova Scotia Health Authority Research Fund"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:p>Cutaneous syncytial myoepithelioma (CSM) is a recently recognized, histopathological variant of myoepithelial (ME) tumors of the skin. It is characterized by a syncytial arrangement of spindled, ovoid, and\/or epithelioid cells forming a well\u2010circumscribed, unencapsulated dermal nodule. There is a paucity of intervening stroma, and absent duct or gland formation. Strong immunohistochemical staining for S100 and epithelial membrane antigen (EMA) has been described, while cytokeratin expression has been uncommon. The majority of CSMs harbor a rearrangement involving the <jats:italic>EWSR1<\/jats:italic> gene. Although various fusion partner genes have been discovered in ME tumors at other anatomic sites, none has yet been described in CSM. We present a case of CSM represented clinically by a papule on the mid\u2010upper back of a healthy 44\u2010year\u2010old female. It exhibited morphological and immunohistochemical features of a CSM with strong, diffuse S100 and alpha\u2010actin expression, and focal positivity for EMA and cytokeratin AE1\/AE3. Fluorescence in\u2010situ hybridization showed an <jats:italic>EWSR1<\/jats:italic> gene rearrangement. Massively parallel next\u2010generation RNA sequencing revealed <jats:italic>PBX3<\/jats:italic> as the fusion partner. The <jats:italic>EWSR1\u2010PBX3<\/jats:italic> gene fusion has been previously identified in three cases of ME tumors of bone and soft tissue, and in a case of retroperitoneal leiomyoma. This is the first report of an <jats:italic>EWSR1\u2010PBX3<\/jats:italic> fusion in CSM.<\/jats:p>","DOI":"10.1111\/cup.13450","type":"journal-article","created":{"date-parts":[[2019,3,5]],"date-time":"2019-03-05T03:59:03Z","timestamp":1551758343000},"page":"421-424","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["<i>EWSR1\u2010PBX3<\/i> gene fusion in cutaneous syncytial myoepithelioma"],"prefix":"10.1111","volume":"46","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7255-2718","authenticated-orcid":false,"given":"William F.","family":"MacKinnon","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Nova Scotia Health Authority  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3964-9790","authenticated-orcid":false,"given":"Michael D.","family":"Carter","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Nova Scotia Health Authority  Halifax Nova Scotia Canada"},{"name":"Department of Pathology Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia A.","family":"Bridge","sequence":"additional","affiliation":[{"name":"Department of Pathology and Microbiology University of Nebraska Medical Center  Omaha Nebraska"},{"name":"Translational Genomics Research Institute (TGen)  Phoenix Arizona"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert D.","family":"Tremaine","sequence":"additional","affiliation":[{"name":"Department of Medicine Nova Scotia Health Authority  Halifax Nova Scotia Canada"},{"name":"Department of Medicine Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9246-4317","authenticated-orcid":false,"given":"Noreen M.G.","family":"Walsh","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Nova Scotia Health Authority  Halifax Nova Scotia Canada"},{"name":"Department of Pathology Dalhousie University  Halifax Nova Scotia Canada"},{"name":"Department of Medicine Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,4,3]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0560.2003.00063.x"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182772bba"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13000-015-0420-z"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000478662"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/cup.13005"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200103000-00009"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.humpath.2003.08.016"},{"key":"e_1_2_5_9_1","unstructured":"JoVY HornickJL FletcherCDM. 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