Cushing’s syndrome (CS) is a potentially fatal disease often caused by the presence of ACTH-secreting PAs, which leads to the onset of Cushing’s disease (CD), or, more rarely, by the existence of either primary adrenal gland disease or ACTH-producing carcinoid tumors, which determine ectopic ACTH secretion (EAS) [65]. This evidence concerns the gene POMC and Cowden syndrome 1.