Primary Bilateral Macronodular Adrenal Hyperplasia

Primary bilateral macronodular adrenal hyperplasia (PBMAH) is increasingly recognized as imaging is widely used, leading to incidental detection in asymptomatic individuals. Adrenal imaging defines radiologic features. All patients should undergo clinical and biochemical assessment for steroid excess-particularly cortisol, as many exhibit mild autonomous cortisol secretion. Genetic mutations are common, especially Armadillo Repeat Containing 5 in the more severe phenotypes and KDM1A in food-dependent cases. Aberrant hormone receptor expression and intra-adrenal adrenocorticotropin production by steroidogenic cells may also contribute to cortisol dysregulation in many patients. Management depends on cortisol status.