Adrenocorticotropic hormone-independent Cushing syndrome

Adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS) accounts for approximately 20% of all endogenous causes and results from autonomous secretion of cortisol from an adrenal gland lesion, usually an adenoma, adrenocortical carcinoma, or, rarely, ACTH-independent macronodular adrenal hyperplasia or primary pigmented nodular adrenal disease. Unless it is a florid presentation, CS can be challenging to diagnose owing to its variable clinical symptoms and signs, most of which are common in the general population. We report clinical presentations and treatment outcomes (major complications) of four patients with ACTH-independent CS.