Preclinical Models of Rare Pediatric Sarcomas: Ewing sarcoma and Ewing-like sarcomas

Abstract

Pediatric sarcomas are extremely rare and effective therapies are often lacking. Research is complicated by the limited availability of tumor material. Therefore, patient-derived preclinical models that recapitulate their original tumor are essential to study effective treatment options and molecular pathways that drive tumor growth. Here, we have established preclinical in vitro models of rare pediatric sarcomas, including Ewing sarcoma and DSRCT, which enabled for drug screening to identify essential molecular pathways and potential therapeutic options. Additionally, we studied the single cell architecture of Ewing sarcoma tumors, which revealed the composition of immune cells, including potential targetable immune checkpoint inhibitors. Together, these studies improve preclinical research of pediatric sarcomas, which can benefit personalized patient care.