{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T09:24:07Z","timestamp":1787131447900,"version":"build-2736575974"},"reference-count":68,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T00:00:00Z","timestamp":1787097600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T00:00:00Z","timestamp":1787097600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Serum response factor (SRF) is a ubiquitously expressed transcription factor that regulates immediate early genes, cytoskeletal organization, and muscle differentiation. Although SRF plays critical roles in development and cell growth, genomic alterations of\n                    <jats:italic>SRF<\/jats:italic>\n                    have long appeared absent in human neoplasms. Recently, however, recurrent\n                    <jats:italic>SRF<\/jats:italic>\n                    gene rearrangements have been identified in a spectrum of soft tissue tumors with myogenic features, including rhabdomyosarcomas, perivascular tumors, inflammatory myofibroblastic tumors, myoepitheliomas and peripheral nerve sheath tumors. This review summarizes current knowledge on\n                    <jats:italic>SRF<\/jats:italic>\n                    fusion genes and their biological and clinical implications. SRF fusions retain the N\u2010terminal DNA\u2010binding domain while replacing the native transactivation domain with that of a partner protein, frequently a transcription factor or co\u2010activator. This structural configuration leads to constitutive activation of SRF and dysregulation of transcriptional programs related to myogenesis, cytoskeleton organization, and, in some cases, inflammatory signaling.\n                    <jats:italic>SRF<\/jats:italic>\n                    \u2010rearranged tumors predominantly affect children, usually display low\u2010grade behavior, and are often cured by complete surgical resection, although rare recurrences and metastases have been reported. Within perivascular myoid neoplasms,\n                    <jats:italic>SRF<\/jats:italic>\n                    \u2010rearranged tumors show consistent phenotypic and clinical features, supporting their recognition as a distinct tumor entity, whereas\n                    <jats:italic>SRF<\/jats:italic>\n                    fusions identified in rhabdomyosarcomas and rare isolated cases likely represent separate biological contexts. Understanding\n                    <jats:italic>SRF<\/jats:italic>\n                    fusion\u2010driven transcriptional dysregulation provides insights into tumorigenesis and may inform future diagnostic and therapeutic strategies in myoid soft tissue neoplasms. \u00a9 2026 The Pathological Society of Great Britain and Ireland.\n                  <\/jats:p>","DOI":"10.1002\/path.70114","type":"journal-article","created":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T08:27:16Z","timestamp":1787128036000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>\n                      <i>SRF<\/i>\n                    <\/scp>\n                    fusion genes in myoid soft tissue tumors"],"prefix":"10.1002","author":[{"given":"Ariane","family":"Sablon","sequence":"first","affiliation":[{"name":"de Duve Institute UCLouvain  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Constance","family":"Pirson","sequence":"additional","affiliation":[{"name":"de Duve Institute UCLouvain  Brussels Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9717-8205","authenticated-orcid":false,"given":"Cristina R","family":"Antonescu","sequence":"additional","affiliation":[{"name":"Department of Pathology Memorial Sloan Kettering Cancer Center  New York NY USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8016-6689","authenticated-orcid":false,"given":"Jean\u2010Baptiste","family":"Demoulin","sequence":"additional","affiliation":[{"name":"de Duve Institute UCLouvain  Brussels 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