Steroidogenesis inhibitors are recommended by the Endocrine Society as second-line treatment after adenomectomy in CD, depending on clinical circumstances; as first-line treatment for patients with ectopic adrenocorticotropic hormone (ACTH) secretion when a tumor is not detected; or as an adjunct treatment for patients with adrenocortical carcinoma [6]. Here, POMC is linked to adrenal cortex carcinoma.