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EWSR1: the promiscuous king of mesenchymal neoplasia
  1. Emily Anne Towery,
  2. David James Papke
  1. Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr David James Papke, Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA; dpapke{at}bwh.harvard.edu

Abstract

EWSR1 is the most commonly rearranged gene in mesenchymal neoplasia, and its myriad chimeric oncoproteins drive widely disparate neoplasms. Here, we survey selected EWSR1 rearrangements, including well-described EWSR1 fusions with CREB family members, ATF1 and CREB1, as well as fusions in emerging entities such as mesenchymal neoplasms with EWSR1::PATZ1 and EWSR1::NFATC2 fusions. We also discuss recent data demonstrating the imperfect specificity of EWSR1::WT1 and, possibly, EWSR1::FLI1 fusions.

  • Pathology, Surgical
  • Sarcoma
  • Soft Tissue Neoplasms

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Footnotes

  • Handling editor Vikram Deshpande.

  • X @EToweryMD, @PapkeDavid

  • Contributors EAT and DJP wrote and edited the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.