Bilateral adrenal Cushing's syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease.
Corticotropin (ACTH)-independent bilateral macronodular adrenal hyperplasia (AIMAH) and primary pigmented nodular adrenocortical disease (PPNAD) are responsible for approximately 10% of adrenal Cushing's syndrome. AIMAH also can be present as subclinical bilateral incidentalomas in sporadic or familial forms. Diverse aberrant hormone receptors have been found to be implicated in the regulation of steroidogenesis and pathophysiology of AIMAH. PPNAD can be found alone or in the context of Carney complex, a multiple endocrine neoplasia syndrome. Additionally, it can be secondary to mutations of type 1 alpha-regulatory subunit of cAMP-dependent protein kinase A (PRKARIA). Strategies for the investigation and treatment of AIMAH and PPNAD are discussed.
Lacroix
André
A
Department of Medicine, Hôtel-Dieu du Centre Hospitalier de l'Université de Montréal, 3840 Saint-Urbain Street, Montreal, Quebec H2W 1T8, Canada. andre.lacroix@umontreal.ca
Bourdeau
Isabelle
I
D000302:Adrenal Cortex / Q000473:pathology / Q000503:physiopathology; D000303:Adrenal Cortex Diseases / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D003480:Cushing Syndrome / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D006965:Hyperplasia