Pheochromocytoma in a patient with end-stage renal disease

Pheochromocytoma is a rare tumor. To our knowledge only 15 cases have been reported in patients with end-stage renal disease (ESRD). We describe a 46-year-old woman with ESRD and a history of paroxysmal and difficult-to-control hypertension. During anesthesia for a surgical procedure, the patient experienced blood pressure lability with systolic blood pressures ranging from 76 to 360 mm Hg. Serum catecholamine concentrations were 2,698 pg/ mL (reference value, <750 pg/mL) for norepinephrine, 33 pg/mL (<110 pg/mL) for epinephrine, and 55 pg/mL (<30 pg/mL) for dopamine. The concentrations of plasma metanephrines were 6.84 nmol/L (<0.50 nmol/L) for metanephrine and 14.64 nmol/L (<0.90 nmol/L) for normetanephrine. Abdominal computed tomography showed a right-sided, 4-cm mass posterior to the infrahepatic inferior vena cava. Following blood pressure control with alpha- and beta-adrenergic blockade, the mass was removed. Pathologic examination demonstrated the mass was a pheochromocytoma. The maximum postoperative systolic blood pressure was 160 mm Hg. Postoperative plasma normetanephrine concentration was 2.80 nmol/L, and metanephrine was obscured by interfering substances. This case report and literature review emphasizes the difficulty in diagnosing pheochromocytomas in patients with ESRD despite the myriad of available diagnostic tests.