Giant Cystic Pheochromocytoma Containing High Concentrations of Catecholamines and Metanephrines

Abstract

A 27-yr-old woman, known with neurofibromatosis type 1 and generalized anxiety disorder, presented with headaches, episodic palpitations, and pallor. Atenolol, started for hypertension by her family doctor, triggered a hypertensive crisis (226/126 mm Hg). Twentyfour-hour urine analysis demonstrated elevated excretion of metanephrine [65.7 ( 1.52; upper limit of reference range) mol/d] and normetanephrine [59.27 ( 1.95) mol/d], and to a lesser extent norepinephrine [2806 ( 569) nmol/d] and epinephrine [1222 ( 149) nmol/d]. Abdominal computed tomography (CT) scan showed a large solid and cystic lesion involving the right adrenal gland (Fig. 1A). 131-I methyliodobenzylguanidine scan showed a right adrenal tumor with prominent peripheral uptake and large central defect (Fig. 1B). After medical preparation with 3 wk of phenoxybenzamine (up to 90 mg/d), propranolol, and preoperative saline loading, the patient appeared clinically adequately blocked. However, anesthetic induction (fentanyl, propofol, lidocaine, and rocuronium) caused a hypertensive crisis (280/170 mm Hg) and prevented surgery. Phenoxybenzamine dosage was increased to 60 mg three times a day, and nifedipine SR 30 mg was added. Intravenous phenylephrine (1 mg) did not affect intraarterial blood pressure, indicating adequate -blockade. The next day, during open adrenalectomy, brittle blood pressure was noted until completion of tumor dissection. A sample of the liquefied tumor center was obtained (Fig. 2). Compared with plasma reference values (1), the cystic fluid contained extremely high concentrations of norepinephrine [65,967 ( 3.4) nmol/liter], epinephrine [51,000 ( 0.8) nmol/liter], dopamine [791.5 ( 0.21) nmol/liter], metanephrine [1,150 ( 0.3) nmol/liter], and normeta-