What specialist should be referred for a functioning adrenal adenoma?

Specialist Referral for Functioning Adrenal Adenoma

Patients with functioning adrenal adenomas should be referred to an endocrinologist for initial evaluation and hormonal management, with subsequent involvement of an endocrine surgeon (or urologist with adrenal expertise) when surgical intervention is indicated. 1

Multidisciplinary Team Approach

A low threshold should exist for multidisciplinary review involving endocrinologists, surgeons, and radiologists when there is evidence of hormone hypersecretion or when adrenal surgery is being considered. 1 This collaborative approach ensures optimal management decisions regarding:

• Confirmation and characterization of hormonal excess 1
• Assessment of surgical candidacy 1
• Selection of appropriate surgical approach 1

Endocrinologist Role

The endocrinologist should be the initial specialist consulted for functioning adrenal adenomas to:

• Complete comprehensive hormonal evaluation including 1 mg dexamethasone suppression testing for cortisol excess, aldosterone-to-renin ratio for primary aldosteronism (if hypertension/hypokalemia present), and plasma/urinary metanephrines for pheochromocytoma 1
• Screen for and manage cortisol-related comorbidities such as hypertension, type 2 diabetes, osteoporosis, and metabolic complications that may be attributable to autonomous cortisol secretion 2, 3
• Provide preoperative medical optimization for specific functional tumors (e.g., alpha-blockade for pheochromocytoma, potassium repletion and blood pressure control for aldosteronoma) 1
• Coordinate postoperative hormone replacement when needed, particularly for cortisol-secreting adenomas requiring glucocorticoid supplementation until hypothalamic-pituitary-adrenal axis recovery 1

Endocrine Surgeon/Urologist Role

Surgical referral is indicated for:

• All cortisol-secreting adenomas with clinically apparent Cushing's syndrome requiring unilateral adrenalectomy, preferably via minimally invasive surgery when feasible 1
• All aldosterone-secreting adenomas confirmed by adrenal vein sampling to demonstrate lateralization, requiring unilateral adrenalectomy 1
• All pheochromocytomas requiring adrenalectomy with appropriate preoperative alpha-blockade 1
• Younger patients with mild autonomous cortisol secretion (post-dexamethasone cortisol 51-138 nmol/L) who have progressive metabolic comorbidities attributable to cortisol excess, after shared decision-making 1, 2

Surgical Approach Considerations

Minimally invasive adrenalectomy should be performed when feasible for functioning adenomas that can be safely resected without tumor capsule rupture 1 Laparoscopic adrenalectomy is the preferred approach for benign functioning tumors including aldosteronomas and most cortisol-secreting adenomas. 1

Critical Pitfall to Avoid

Never proceed with surgery or biopsy before definitively excluding pheochromocytoma, as failure to screen for catecholamine excess can be fatal. 4, 5 All patients with adrenal masses >10 HU on non-contrast CT or any symptoms of adrenergic excess require pheochromocytoma screening with plasma or 24-hour urinary metanephrines before any intervention. 1

Special Considerations for Specific Functional Adenomas

Aldosterone-Secreting Adenomas

Adrenal vein sampling is mandatory prior to offering adrenalectomy in patients with confirmed primary aldosteronism to distinguish unilateral adenoma from bilateral hyperplasia. 1 This is critical because only unilateral disease benefits from surgery, while bilateral hyperplasia requires medical management with mineralocorticoid receptor antagonists (spironolactone or eplerenone). 1

Cortisol-Secreting Adenomas

Patients require assessment of autonomous cortisol secretion severity:

• Post-dexamethasone cortisol ≤50 nmol/L (≤1.8 µg/dL) excludes autonomous secretion 2, 3
• Post-dexamethasone cortisol >138 nmol/L (>5.0 µg/dL) indicates autonomous cortisol secretion requiring surgical consideration 2, 3
• Post-dexamethasone cortisol 51-138 nmol/L represents mild autonomous cortisol secretion (MACS) requiring individualized surgical decision-making based on age and comorbidity burden 1, 2