Two comprehensive genomic analyses including the TCGA dataset identified alterations in CTNNB1, TP53, CDKN2A, RB1 and MEN1—genes that had previously been reported as mutated in ACC—as well as in ZNRF3, DAXX, TERT and MED12 [8, 16]. This evidence concerns the gene ZNRF3 and adrenal cortex carcinoma.