POMC and ACTH Syndrome, Ectopic: It has been claimed that 2.5–5 % of all ectopic ACTH syndromes is caused by a pheochromocytoma [5, 10], while we found ACTH secretion in 10 % from medullary cells in a pheochromocytoma and 10 % from adrenal medullary hyperplasia.