Pheochromocytoma: diagnosis and management update

Abstract

Pheochromocytoma is a rare but extremely treacherous neuroendocrine tumor causing manifestations by secreting catecholamines into the circulation. It is usually fatal if unrecognized. Manifestations can mimic many diseases and cause erroneous diagnoses. Approximately 15% are malignant, 18% extra-adrenal, and 20% familial. Plasma or urinary metanephrines are approximately 98% sensitive for detecting pheochromocytomas. They can be localized by MRI and CT; (131)I- and (123)I-metaiodobenzylguanidine (MIBG) are highly specific and 81% to 90% sensitive, respectively. Suspect pheochromocytoma in patients with sustained or paroxysmal hypertension or in normotensives having manifestations of hypercatecholaminemia. Surgical removal is usually curative; chemotherapy and radiotherapy are palliative for malignant pheochromocytomas.