The promoter activity of IGF-1R is significantly activated by the binding of EWS-WT1 with the -331 to -40 region of the IGF-1R promoter in desmoplastic small round cell tumor (DSRCT), a malignant soft tissue sarcoma occurring in young children (Karnieli et al., 1996). Here, EWSR1 is linked to desmoplastic small round cell tumor.