Extra-adrenal pheochromocytoma presenting with life-threatening ventricular tachycardia: a case report.

Extra-adrenal pheochromocytoma develops in paraganglion chromaffin cells of the sympathetic nervous system. It probably represents at least 15% of adult and 30% of childhood pheochromocytomas. Although electrocardiographic abnormalities occur in up to 75% of patients with pheochromocytoma, there are only three other reported cases of pheochromocytoma that initially presented with ventricular tachycardia. In this report, we describe a 64-year-old woman with an abdominal para-aortic extra-adrenal pheochromocytoma who presented with pulseless ventricular tachycardia and needed electrical defibrillation initially to keep vital signs. The 24-hour urine vanillylmandelic acid was 35.8 mg. Computerized tomography showed a left retroperitoneal mass. The postoperative course was uneventful. Pheochromocytoma was proved by histologic study.