The ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome (CS), an occasional manifestation of NETs, is caused most commonly by small cell lung carcinomas, following by bronchial carcinoid tumors, thymic carcinoids, islet cell tumor of pancreas, medullary thyroid carcinoma, and rarely pheochromocytoma [2]. This evidence concerns the gene POMC and Cowden syndrome 1.