The Matrix Reloaded: Modeling, Remodeling, and Silencing in Desmoplastic Small Round Cell Tumor (Conference Abstract)

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare and aggressive pediatric sarcoma defined by the EWSR1::WT1 fusion and a dense desmoplastic extracellular matrix (ECM) that promotes tumor growth while impairing drug delivery and immune infiltration. The lack of physiologically relevant in vitro models and validated therapeutic targets hampers investigation of ECM-DSRCT interaction and therapeutic response. Herein, we established a 3D organotypic DSRCT model and explored the therapeutic potential of fusion silencing through LNP delivery.