POMC and Cowden syndrome 1: In about 70% of cases, CS is caused by an adrenocorticotropic hormone (ACTH)-producing pituitary tumor (Cushing’s disease, CD), while a primary adrenal hyperfunction (ACTH-independent CS, ACS) and ectopic ACTH-secreting neuroendocrine tumor (EAS) account for the remaining 30% of cases [1].