Insulin-Like Growth Factor Signaling in Pediatric Sarcomas

Uncontrolled cell growth is central to carcinogenesis. Growth factors, as well as growth factor-initiated pathways, that regulate cell growth control and also modulate cell phenotype are undoubtedly important to the etiology of cancer. Research on cancer biology and cellular signaling mediated by the family of insulin/insulin-like growth factor (IGF) receptors and their cognate ligands have been ongoing for over three decades. The excitement and attention focused on the IGF system has recently turned to targeting insulin-like growth factor receptor 1 (IGF-1R) using humanized and fully human antibodies. The use of this approach in clinical trials has resulted in several dramatic responses in patients with recurrent and refractory pediatric sarcomas. This chapter focuses on highlights in the biological, therapeutic, preclinical, and clinical roles of the IGF family in pediatric sarcomas.