However, the most common molecular alterations found in ductal adenocarcinoma (KRAS), cystic neoplasms (GNAS and RNF43), and NENs (MEN1, DAXX, and ATRX) are rarely found in ACC [1–4, 8, 12, 13, 17]. Here, RNF43 is linked to adrenal cortex carcinoma.