Abstract
Pheochromocytomas and functional paragangliomas are catecholamine-producing tumors presenting with various clinical symptoms, but mostly with headache, sweating, palpitations, and hypertension. Biochemical testing for pheochromocytoma should be performed not only in symptomatic subjects or in subjects with adrenal incidentaloma but also in subjects with a genetic predisposition for pheochromocytoma (multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome, neurofibromatosis type 1 and mutations of succinate dehydrogenase genes).
Once a pheochromocytoma is proven, computed tomography, magnetic resonance imaging and functional imaging with [123I]-metaiodobenzylguanidin may be used for tumor localization. Adequate medical pre-treatment is essential for successful operation which is performed in most cases by minimal invasive surgery.
After tumor removal, further follow-up is necessary due to possible recurrence.