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We report three cases of a unique soft tissue tumor in young children (5\u2009months to 2\u2009years old) displaying a purely fibromyxoid histology, with positive staining for desmin and CD34. In two cases, RNA sequencing detected a <jats:italic>YWHAZ<\/jats:italic>\u2010<jats:italic>PLAG1<\/jats:italic> gene fusion, while in the third case, a previously unreported <jats:italic>EEF1A1<\/jats:italic>\u2010<jats:italic>PLAG1<\/jats:italic> fusion was identified. <jats:italic>PLAG1<\/jats:italic> fusions have been reported in several pathologic entities including pleomorphic adenoma, myoepithelial tumors of skin and soft tissue, and lipoblastoma, the latter occurring preferentially in young children. In these tumors, expression of a full length PLAG1 protein comes under the control of the constitutively active promoter of the partner gene in the fusion, and the current cases conform to that model. Overexpression of PLAG1 was confirmed by diffusely positive immunostaining for PLAG1 in all three cases. Our findings raise the possibility of a novel fibromyxoid neoplasm in childhood associated with these rare <jats:italic>PLAG1<\/jats:italic> fusion variants. The only other report of a <jats:italic>PLAG1\u2010YWHAZ<\/jats:italic> fusion occurred in a pediatric tumor diagnosed as a \u201cfibroblastic lipoblastoma.\u201d This finding raises the possibility of a relationship with our three cases, even though our cases lacked any fat component. Further studies with regard to a shared pathogenesis are required.<\/jats:p>","DOI":"10.1002\/gcc.22926","type":"journal-article","created":{"date-parts":[[2020,12,10]],"date-time":"2020-12-10T21:49:07Z","timestamp":1607636947000},"page":"263-271","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Pediatric fibromyxoid soft tissue tumor with <scp><i>PLAG1<\/i><\/scp> fusion: A novel entity?"],"prefix":"10.1002","volume":"60","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7430-761X","authenticated-orcid":false,"given":"Catherine T.","family":"Chung","sequence":"first","affiliation":[{"name":"Division of Pathology The Hospital for Sick Children  Toronto Canada"},{"name":"Department of Laboratory Medicine and Pathobiology University of Toronto  Toronto Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9717-8205","authenticated-orcid":false,"given":"Cristina 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