Alteration of the Insuline-Like Growth Factor-2 (IGF2) gene is a hallmark of ACC (loss of heterozigosity in 90% of cases) but overexpression of the corresponding insulin-like growth factor-2/receptor-1 (IGF2/IGF1R) axis seems not to be a driver pathway in ACC as demonstrated by failure of clinical intervention with the anti-IGF2/IGF1R linsitinib [18]. The gene discussed is IGF2; the disease is adrenal cortex carcinoma.