(CSEMP036) A CASE OF HYPERTENSIVE URGENCY DURING BILATERAL ADRENAL ABLATION FOR ECTOPIC CUSHING SYNDROME

Abstract:

Background: Ectopic Cushing syndrome is a disorder characterized by cortisol excess due to production of adrenocorticotropic hormone (ACTH) by a non-pituitary tumour. When resection of the ACTH-producing tumour or adrenalectomy are contraindicated, and medical therapy is unsuccessful, bilateral adrenal ablation may be considered. However, this may result in hypertensive urgency due to systemic catecholamine release. There are no current guidelines to direct whether patients with a negative pheochromocytoma workup undergoing adrenal ablation should be pre-treated with alpha- and beta-blockade with the aim of preventing intraprocedural hypertension.

Case Presentation: A 61-year-old man admitted to hospital for cellulitis was diagnosed with non-small cell lung cancer (NSCLC). During his admission, he had refractory hypertension (systolic blood pressure [SBP] 150-160s mmHg) and hypokalemia (2.2-3.3 mmol/L) despite aggressive medical management. He was diagnosed with ACTH-dependent Cushing syndrome based on an elevated ACTH (73.7 pmol/L), 24-hour urine cortisol (4514 nmol/day) and non-suppression with a 1 mg dexamethasone suppression test (DST) (serum cortisol 2008 nmol/L). Ectopic ACTH production was likely caused by NSCLC. The patient was not a surgical candidate and ketoconazole was contraindicated given elevated transaminases (ALT 410 U/L), so bilateral microwave adrenal ablation was performed. Prior to ablation, 24-hour urine fractionated metanephrines were normal. He received pre-treatment with doxazosin (maximum dose 2 mg BID) and bisoprolol (maximum dose 10 mg daily). During ablation of the right adrenal gland, his SBP increased to the high 200s mmHg. During ablation of the left, maximum SBP was 315 mmHg leading to a pause of ablation before completion. He did not require intraprocedural anti-hypertensive medications. The adrenal ablation was successful, and his serum cortisol decreased from 2016 nmol/L pre-ablation to 538 nmol/L post-ablation. He was placed on a hydrocortisone taper and off anti-hypertensive agents. Ten days following ablation, he developed hypotension and suffered a cardiac arrest related to massive gastrointestinal bleeding and passed away shortly after.

Discussion: This case demonstrates that hypertensive urgency during adrenal ablation for ectopic Cushing syndrome can occur even with pre-treatment with alpha- and beta-blockade. It is important that guidelines are developed for the prevention and management of hypertensive urgency in this population.