For example, a hotspot somatic mutation in the PKA catalytic subunit alpha gene (PRKACA) has been identified in ACA (14), germline, and somatic mutations of armadillo repeat containing 5 gene (ARMC5) have been described in patients with primary bilateral macronodular adrenal hyperplasia (PBMAH) (15), and somatic alterations in the E3 ubiquitin ligase gene ZNRF3 were recently identified in ACC (16). This evidence concerns the gene ZNRF3 and adrenal cortex carcinoma.