POMC and Cowden syndrome 1: Approximately 80% of all endogenous CS cases are caused by corticotrophin (ACTH) hypersecretion (ACTH-dependent CS), of which 90% are represented by corticotroph pituitary adenomas (Cushing’s disease, CD), while ectopic ACTH syndrome (EAS) occurs in about 10% of cases [1, 2].