Germline and somatic mutations in the TP53 (tumor protein p53) gene [13,14], activation of Wnt/ß-catenin pathway through somatic CTNNB1 (catenin beta 1) mutations [15], overexpression of IGF-2 (insulin-like growth factor 2) [16,17] and SF1 (steroidogenic factor-1) genes [18], and mutations in mismatch repair genes [19] are all related to ACC formation. This evidence concerns the gene IGF2 and adrenal cortex carcinoma.