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Alyse Goldberg, Stephen E. Pautler, Christopher Harle, Alan Dennis, Irina Rachinsky, Achal Dhir, Shawna Boyle, Vivian McAlister, Stan Van Uum, Giant Cystic Pheochromocytoma Containing High Concentrations of Catecholamines and Metanephrines, The Journal of Clinical Endocrinology & Metabolism, Volume 96, Issue 8, 1 August 2011, Pages 2308–2309, https://doi.org/10.1210/jc.2011-0465
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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). Twenty-four-hour urine analysis demonstrated elevated excretion of metanephrine [65.7 (<1.52; upper limit of reference range) μmol/d] and normetanephrine [59.27 (<1.95) μmol/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 α-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 normetanephrine [2,400 (<0.6) nmol/liter]. Similar findings have previously been reported for catecholamines (2).
