Sarcoma Diagnosis and Treatment
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The data suggest that, except for SRF::PDGFRB, SRF fusions belong to the family of oncogenes acting as hyperactivated transcription factors in human myoid soft tissue tumors.
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ABSTRACT SRF fusion genes drive the pathogenesis of muscle-related soft tissue tumors, including subsets of perivascular tumors, inflammatory myofibroblastic tumor, and rhabdomyosarcoma. SRF encodes Serum Response Factor, a well-characterized transcription factor that regulates muscle development. We characterized four fusion genes: SRF::RELA, SRF::FOXO1, SRF::ICA1L, and SRF::PDGFRB. All localized to the nucleus and dimerized through the SRF MADS box. SRF::RELA, SRF::FOXO1, and SRF::ICA1L acted as constitutively active transcription factors independent of canonical cofactors, binding SRF target promoters and driving transcription via the partner transactivation domain (TAD). A cryptic TAD was uncovered in ICA1L. These fusions promoted mesenchymal cell growth and upregulated muscle-related genes in mesenchymal stem cells, recapitulating transcriptional signatures of human tumors. In contrast, SRF::PDGFRB acted through its kinase domain, was imatinib-sensitive, activated STAT1 and stimulated inflammation genes, consistent with its tumor phenotype. SRF fusions thus define a novel family of oncogenes in human myoid soft tissue tumors. Graphical Abstract
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@article{Pirson2026fusion,
title = {SRF fusion oncogenes encode constitutively activated chimeric transcription factors in myoid soft tissue tumors},
author = {Constance Pirson and Ariane Sablon and Axelle Loriot and Pierre Autin and Koen Braat and Marie Karanian and Cristina R. Antonescu and Franck Tirode and Jean‐Baptiste Demoulin},
journal = {Cellular and Molecular Life Sciences},
year = {2026},
doi = {10.1007/s00018-026-06382-z},
url = {https://doi.org/10.1007/s00018-026-06382-z}
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