title: Recent advances in targeted therapy for Ewing sarcoma.
abstract: Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.
authors.affiliation: Cancer Therapeutics Laboratory, Center for Personalized Cancer Medicine, Discipline of Medicine, University of Adelaide, Adelaide, SA, Australia; Center for Childhood Cancer and Blood Disorders, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Kathleen I
authors.initials: KI
authors.lastname: Pishas
authors.affiliation: Center for Childhood Cancer and Blood Disorders, The Research Institute at Nationwide Children's Hospital, Columbus, OH, USA; Division of Pediatric Hematology/Oncology/Bone Marrow Transplant, Ohio State University, Columbus, OH, USA.
authors.forename: Stephen L
authors.initials: SL
authors.lastname: Lessnick
keywords: EWS/FLI; Ewing sarcoma; ganitumab; sarcoma