A growing body of evidence suggests that genetic factors may predispose to ACC development, such as overexpression of insulin-like growth factor 2 (IGF2), mutations in TP53, catenin beta-1 (CTNNB1), and the Wnt/β-catenin signaling pathway, particularly zinc and ring finger 3 (ZNRF3) in adults [11,12,13,14]. This evidence concerns the gene CTNNB1 and adrenal cortex carcinoma.