POMC and classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: In addition, patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, a condition which is associated with chronically high plasma ACTH levels, can present with adrenal myelolipoma (67–69), suggesting a role of ACTH in the development of lipomatous tissue inclusions in the adrenal glands.