ACTH-independent CS (15–20%) results from excessive secretion of cortisol by adrenocortical tumors, classified as either adenoma (10%) or carcinoma (8%), by primary pigmented nodular adrenocortical disease (PPNAD, 1%) or by ACTH-independent bilateral macronodular adrenocortical hyperplasia (AIMAH, <1%) [3, 6–8]. Here, POMC is linked to carcinoma.