Convincing preclinical evidence supporting the efficacy of IGF1R pathway inhibition in the treatment of cancer has led to the development of many IGF1R pathway inhibitors [2,3,4,5,6], which have been investigated in numerous clinical trials in breast cancer; Ewing sarcoma and various other types of solid tumors, like non-small cell lung cancer, hepatocellular, gastric and esophageal carcinoma (Table 1) [7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26]. Here, IGF1R is linked to Ewing sarcoma.