Diagnosis and management of pheochromocytoma - recent advances and current concepts

Pheochromocytoma is a rare but extremely treacherous neuroendocrine tumor, usually occurring in the adrenals but sometimes elsewhere in the abdomen, pelvis, chest, neck, and head. It causes manifestations by secreting catecholamines into the circulation. Tragically, up to 50% of pheochromocytomas are discovered at autopsy, mainly because this tumor was not considered. Clinicians must think of pheochromocytoma whenever any manifestations suggesting hypercatecholaminemia occur. Manifestations can mimic many other diseases, but manifestations without sustained or paroxysmal hypertension are rarely due to pheochromocytoma. However, familial pheochromocytoma, which comprises about 30% of tumors, may rarely be asymptomatic and cause no hypertension. Biochemical testing can almost always establish the presence or absence of a pheochromocytoma. Tumor localization with magnetic resonance imaging, computed tomography, or 131I or 123I-MIBG is nearly always possible. Surgical removal is usually curative; chemotherapy and radiotherapy are palliative for malignant pheochromocytoma.