Board Review

A 20-year-old woman is admitted to the hospital for an elective adrenalectomy. Her history is significant for recent onset of hypertension, weight gain, generalized weakness, and easy bruising. Laboratory evaluation demonstrated an elevated urine free cortisol and lack of suppression of serum cortisol following a low-dose overnight dexamethasone suppression test. Subsequent testing revealed a suppressed adrenocorticotropic hormone level.

A contrast-enhanced adrenal CT scan revealed a well-circumscribed 3.7-cm right adrenal mass with a contrast washout of greater than 50% at 10 minutes.

Which of the following is the most appropriate perioperative management?

A) Postoperative hydrocortisone

B) Postoperative mitotane

C) Postoperative norepinephrine

D) Preoperative phenoxybenzamine

Answer: A - Postoperative hydrocortisone

Objective: Manage a patient with Cushing syndrome following adrenalectomy.

Key Point

Following adrenalectomy, patients with adrenal Cushing syndrome should be treated with stress-dose glucocorticoids during the postoperative period to avoid the risk of acute hypocortisolism.

The most appropriate management of this patient undergoing right adrenalectomy for Cushing syndrome (CS) is postoperative hydrocortisone. The patient has adrenocorticotropic hormone (ACTH)-independent CS, and a contrast-enhanced adrenal CT scan demonstrated a right adrenal mass with imaging characteristics consistent with a benign adenoma. Adrenocortical adenomas typically have low attenuation on unenhanced CT scan (density less than 10 Hounsfield units) and exhibit rapid washout of intravenous iodine contrast media (>50% at 10 minutes). Following adrenalectomy, patients with adrenal CS may develop acute adrenal failure because of hypothalamic-pituitary-adrenal (HPA) axis suppression and contralateral adrenal atrophy. All patients should be treated with stress-dose glucocorticoids and tapered to physiologic replacement until HPA axis recovery is confirmed. Most patients have adrenal insufficiency lasting as long as 12 months.

Postoperative administration of mitotane, an adrenolytic drug, is recommended as adjuvant therapy for patients with locally persistent or metastatic adrenocortical carcinoma (ACC). In these patients, mitotane is associated with objective remissions in approximately 25% of patients. This patient's CT scan findings are not consistent with ACC. Adrenal cancers are typically large (>4-6 cm) with irregular margins and areas of necrosis or calcification. Unenhanced CT will demonstrate high attenuation (density >10 Hounsfield units), and washout of intravenous iodine contrast media is less than 50% at 10 minutes.

Postoperative norepinephrine is not indicated. If the patient were to experience hypotension or shock postoperatively, treatment with vasopressors in addition to glucocorticoid replacement would be considered; however, this would not replace the administration of glucocorticoids in this population at risk for acute hypocortisolism.

Preoperative phenoxybenzamine is indicated in the management of patients with pheochromocytoma, not CS. The purpose of preoperative α-blockade is to provide blood pressure control and decrease the risk of cardiovascular complications related to excessive catecholamine release during intraoperative manipulation of the tumor.

Di Dalmazi G, Berr CM, Fassnacht M, et al. Adrenal function after adrenalectomy for subclinical hypercortisolism and Cushing's syndrome: A systematic review of the literature. JCEM. 2014;99:2637-2645. Link Out