Modeling extracellular matrix-tumor interactions in desmoplastic small round cell tumor: development of a new organotypic model (Conference Abstract)

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare, aggressive soft tissue sarcomaprimarily affecting the peritoneal cavity of children and young adults. It is defined by aspecific chromosomal translocation t(11;22)(p13;q12), that leads to the aberranttranscription factor EWSR::WT1, and a rich desmoplastic extracellular matrix (ECM) thatsupports tumor growth while hindering drug delivery and immune infiltration. Currently,there are no 3D DSRCT in vitro models, which are critical to evaluate treatment strategies.Moreover, it is crucial to identify potential therapeutic targets. Nucleolin (NCL), describedby our group as a safe and specific therapeutic target when expressed on the cell surface(doi: 10.1016/j.nantod.2021.101095), remains unexplored in DSRCT. Herein, we develop anovel organotypic DSRCT model that closely mimics the ECM-tumor dynamics to studytumor progression and NCL-targeted therapies.