ACC is associated with somatic mutations in CTNNB1, APC, MEN1, BRCA1, BRCA2, and PRKAR1A, similar to those in adrenocortical adenoma or hyperplasia, as well as in NF1, NF2, RB1, CDC27, SCN7A, SDK1, TERT, ZNRF3, ATRX, NOTCH1, CIC, KDM6A, and RPL22 genes [158,159,160]. The gene discussed is RB1; the disease is adrenal cortex carcinoma.