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This report describes the first molecularly confirmed LGFMS of the floor of the mouth demonstrating a\n                      <jats:italic>FUS::CREB3L2<\/jats:italic>\n                      gene fusion.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>An 18-year-old male presented with a painless floor of mouth mass of 1-year duration that was clinically suspected to represent a ranula. The mass was subjected to excisional biopsy. Histological examination was salient for an infiltrating proliferation of bland spindle cells arranged in whorled patterns within a collagenous stroma with focal myxoid change.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Immunohistochemical analysis revealed diffuse MUC4 expression, narrowing the diagnosis. Targeted RNA sequencing identified a\n                      <jats:italic>FUS::CREB3L2<\/jats:italic>\n                      fusion involving\n                      <jats:italic>FUS exon 6 and CREB3L2 exon 5<\/jats:italic>\n                      , confirming LGFMS. At 1-year follow-up, the patient showed no clinical evidence of disease and no detectable circulating tumor DNA.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights the critical role of molecular testing in the diagnosis of LGFMS and emphasizes the importance of appropriate management strategies, including aggressive local control and long-term surveillance, given the tumor\u2019s risk of late recurrence and distant metastasis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s12105-026-01888-1","type":"journal-article","created":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T11:14:04Z","timestamp":1769771644000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Novel Case of a Molecularly Confirmed Low-Grade Fibromyxoid Sarcoma of the Floor of the Mouth and Literature Review"],"prefix":"10.1007","volume":"20","author":[{"given":"Eugene G.","family":"Bestman","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6634-8470","authenticated-orcid":false,"given":"John K.","family":"Brooks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott D.","family":"Nelson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5286-4562","authenticated-orcid":false,"given":"Ahmed S.","family":"Sultan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9376-5613","authenticated-orcid":false,"given":"Njood","family":"Hawari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha L.","family":"Jeffrey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jettie","family":"Uyanne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix Kyle","family":"Yip","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin","family":"Artis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manando","family":"Nakasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,1,30]]},"reference":[{"key":"1888_CR1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/256280","volume":"2013","author":"K Maretty-Nielsen","year":"2013","unstructured":"Maretty-Nielsen K, Baerentzen S, Keller J, Dyrop HB, Safwat A (2013) Low-grade fibromyxoid sarcoma: incidence, treatment strategy of metastases, and clinical significance of the FUS gene. 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Their presentation has now been corrected. The original article has been corrected.","order":7,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 May 2026","order":8,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Correction","order":9,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"A Correction to this paper has been published:","order":10,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"https:\/\/doi.org\/10.1007\/s12105-026-01906-2","URL":"https:\/\/doi.org\/10.1007\/s12105-026-01906-2","order":11,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed were in accordance with the ethical standards of institutional and\/or research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This case report did not require review by the University Institutional Review Board.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}},{"value":"The featured patient consented for inclusion of the relevant medical records and photographs in this report.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for Publication"}}],"article-number":"18"}}