This $527,904 Cooperative Agreement award from the National Cancer Institute (NCI) under the Cancer Cause and Prevention Research program (CFDA 93.393) supports the Sloan-Kettering Institute for Cancer Research to conduct a multi-year research project focused on Desmoplastic Small Round Cell Tumor (DSRCT), a rare and highly lethal sarcoma affecting adolescents and young adults.
The key objectives are to: 1) Develop the first genetically engineered mouse model of DSRCT to study tumor biology and progression, including the impact of the EWSR1-WT1 fusion gene and ARID1A mutations; 2) Define the landscape of novel oncogenic "neotranscripts" induced by the EWSR1-WT1 fusion; and 3) Determine the role of recurrent ARID1A loss in DSRCT pathogenesis. The research aims to generate new insights into DSRCT mechanisms to aid the identification of more effective, targeted therapeutic strategies for this highly aggressive sarcoma.
Generated 3/25/25, 5:41 AM