Version July 2025
ACTH: corticotropin; MRI: magnetic resonance imaging; UFC: urinary free cortisol.
* Transsphenoidal surgery with adenomectomy leads to an initial remission rate between 75 to 90% in adults and 70 to 90% in children.
¶ Repeat transsphenoidal surgery leads to a cure rate of 60 to 70% in adults and 45 to 50% in children.
Δ Bilateral adrenalectomy may be preferred in this population because it offers certainty of cure and more rapid control, and some options for medical therapy are contraindicated in pregnancy. Alternatives may include exploratory transsphenoidal surgery or some medications.
◊ This represents our preferred approach. However, either corticotroph-directed agents or adrenal steroidogenesis inhibitor monotherapy may be appropriate in this population.
§ Combination therapy entails use of 2 or more agents, usually concurrent use of 2 adrenal steroidogenesis inhibitors or an adrenal steroidogenesis inhibitor with a corticotroph-directed agent.