Pheochromocytoma and Paraganglioma

Abstract

Pheochromocytomas and paragangliomas are tumors of the chromaffin cells of the adrenal medulla and autonomic nervous system, respectively. In the adrenal medulla and sympathetic ganglia, these tumors secrete catecholamines and are a rare cause of secondary hypertension, cardiomyopathy, myocardial infarction, stroke, and death. Patients are screened with plasma metanephrines and the diagnosis may be confirmed with a 24-h urine metanephrine panel. All adrenal masses found incidentally on imaging should be evaluated for pheochromocytoma. Once a biochemical diagnosis is confirmed, the tumor is localized with an anatomic and then a functional imaging study. Patients are typically prepared preoperatively with alpha-blockade and volume resuscitation. Surgical extirpation, whether through open or laparoscopic surgery, is the only option for cure. An increasing number of these tumors are recognized to arise from a germ-line mutation and therefore consideration must be given to appropriate genetic workup. All patients require lifelong surveillance for both recurrence and metastasis.