Current Approach to Pheochromocytoma
October 01, 2006
Pheochromocytomas are tumors of the neural crest-derived chromaffin cells. The hallmark of this rare and fascinating neoplasm is the synthesis and secretion of catecholamines in an unregulated and potentially life-threatening manner. Most pheochromocytomas produce an abundance of norepinephrine. Epinephrine- or dopamine-secreting pheochromocytomas are less common. Pheochromocytomas can also be nonfunctional. Approximately 10% of pheochromocytomas can be categorized as either bilateral, multifocal, extra-adrenal, familial, or malignant; thus, pheochromocytomas are often remembered by medical students as the "10% tumor." Newer reports, however, suggest that pheochromocytomas may be extra-adrenal in up to 30% of cases.[2,3] This brief review will address the diagnosis and management of benign and malignant pheochromocytoma.