EWSR1: the promiscuous king of mesenchymal neoplasia

Abstract

EWSR1is the most commonly rearranged gene in mesenchymal neoplasia, and its myriad chimeric oncoproteins drive widely disparate neoplasms. Here, we survey selectedEWSR1rearrangements, including well-describedEWSR1fusions with CREB family members,ATF1andCREB1, as well as fusions in emerging entities such as mesenchymal neoplasms withEWSR1::PATZ1andEWSR1::NFATC2fusions. We also discuss recent data demonstrating the imperfect specificity ofEWSR1::WT1and, possibly,EWSR1::FLI1fusions.