A Dilemma in Pre-Operative Management in Normotensive Pheochromocytoma

Presentation Number: SUN 361
Date of Presentation: April 2nd, 2017

Linda Nguyen*1, Kimberly Kochersperger Lessard2 and Nissa Blocher1
1Albert Einstein Medical Center, Philadelphia, PA, 2Einstein Medical Center, Philadelphia, PA


Background:Catecholamine-secreting tumors are rare neoplasms with an annual incidence of 0.8 per 100,000 person-years. Interestingly, 5-15% may be normotensive, rather than hypertensive. In most cases of suspected pheochromocytoma, patients are prepared preoperatively for adrenalectomy with alpha- followed by beta-adrenergic blockade to ensure hemodynamic stability. Management of normotensive patients, however, is controversial.

Case:A 53 year old female with a history of transient ischemic attack, atrial septal defect repair and atrial fibrillation treated with metoprolol presented with one year of intermittent palpitations, headaches, and diaphoresis. She had no history of hypertension and had no elevated blood pressure measurements on record. Initial work up was significant for 24-hour urine epinephrine of 113mcg/24 hours (reference: 0-20) and 24 hours fractionated metanephrine of 2853 mcg/24 hours (reference:35-460). She was confirmed on computed tomography of the abdomen to have a right adrenal mass of 3.3 x 2.9 x 3.9 cm with 38 Hounsfield units on unenhanced images. Robotic-assisted adrenalectomy was scheduled, but pre-operative medical management remained unclear. The patient was maintained on metoprolol for atrial fibrillation and tolerated only a low dose of phenoxybenzamine pre-operatively. Intra-operatively, she developed significant hypertension requiring nitroglycerin infusion. Her blood pressure normalized rapidly following tumor removal. Subsequent pathology confirmed the mass to be a pheochromocytoma. She was discharged in stable condition on post-operative day two and reported complete resolution of her symptoms at follow up.

Discussion: Existing guidelines recommend pre-operative alpha-adrenergic blockade for surgical resection of suspected pheochromocytomas without consideration of hypertensive status. Current literature found similar rates of peri-operative hemodynamic instability in hypertensive and normotensive patients, suggesting similar preparation be used. However, normotensive patients may not tolerate the preparation due to risk of subsequent hypotension. Shao et al found no benefit of pre-operative alpha-adrenergic blockade in normotensive pheochromocytoma. In fact, they found a greater need for intra-operative vasoactive drugs in normotensive patients prepared with alpha blockers versus those not on alpha-blockers. Thus, patients with normotensive pheochromocytoma may have better peri-operative outcomes without the currently recommended pre-operative preparation of alpha-adrenergic blockade.


Nothing to Disclose: LN, KKL, NB