Abstract

CIC::DUX4 translocation-positive sarcomas (CDS) are rare, highly proliferative tumors associated with chemotherapy resistance. Without a clear understanding of the molecular pathways driving CDS, no effective treatment regimens have been developed. Here, we identify protection against DNA damage through upregulation of POLE and DNA repair pathways specific to CDS. These data may explain the high proliferation rate and chemotherapy resistance of these tumors and suggest targeted treatment strategies for CDS.

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