Treatment of Bilateral Adrenal Hyperplasia

Initial Diagnostic Workup

The American Urological Association recommends screening for congenital adrenal hyperplasia by measuring 17-hydroxyprogesterone levels in all patients presenting with bilateral adrenal masses 2
The Endocrine Society suggests assessing for primary aldosteronism with plasma aldosterone concentration, plasma renin activity, and aldosterone-to-renin ratio (typically >30 in Conn syndrome) 3
The American College of Endocrinology recommends evaluating for Cushing's disease by measuring ACTH levels—elevated ACTH indicates pituitary or ectopic sources driving adrenal hyperplasia rather than primary adrenal pathology 1
The National Comprehensive Cancer Network advises ruling out metastatic disease if the patient has a known malignancy elsewhere, as this would fundamentally change management 2

The American Heart Association recommends medical management with mineralocorticoid receptor antagonists as first-line therapy for primary aldosteronism, with spironolactone or eplerenone as treatment options 1

The American College of Surgeons suggests considering bilateral adrenalectomy for severe, refractory Cushing's disease or life-threatening emergencies requiring immediate cortisol control 1
The Endocrine Society recommends lifelong glucocorticoid and mineralocorticoid replacement after bilateral adrenalectomy 1

The Pituitary Society recommends transsphenoidal resection of the pituitary adenoma as the definitive treatment for Cushing's disease, which addresses the root cause of adrenal hyperplasia 1
The American College of Endocrinology suggests medical management with adrenostatic agents if the pituitary tumor is unresectable 1

The American Urological Association advises against performing bilateral adrenalectomy for primary aldosteronism without first attempting adequate medical therapy, as two-thirds of primary aldosteronism cases are bilateral hyperplasia requiring medical rather than surgical management 2
The Endocrine Society recommends against assuming all bilateral adrenal hyperplasia is the same etiology, and failure to screen for congenital adrenal hyperplasia with 17-hydroxyprogesterone can lead to inappropriate treatment 2
The American College of Endocrinology suggests against overlooking the possibility of unilateral autonomous function developing within bilateral hyperplasia, and repeat adrenal venous sampling may reveal lateralization amenable to unilateral adrenalectomy 1
The American College of Surgeons advises against forgetting lifelong hormone replacement counseling before bilateral adrenalectomy, as patients must understand the permanent need for glucocorticoid and mineralocorticoid replacement and the risk of adrenal crisis 1