Abstract
Pheochromocytomas and functional paragangliomas are catecholamine-producing tumors which present typical paroxysmal symptoms. Biochemical testing for pheochromocytoma is performed by using measurement of metanephrines in either plasma or urine not only in subjects with clinical suspicion of pheochromocytoma but also in subjects with incidentally discovered adrenal mass or in subjects with genetic predisposition for pheochromocytoma and paraganglioma.
To localize pheochromocytoma, computed tomography (less frequently magnetic resonance imaging) and functional imaging with [123I]-metaiodobenzylguanidine are used. Medical pre-treatment is essential for successful tumor removal which is performed mostly by minimal invasive surgery.
Long-term follow-up is mandatory due to possible tumor recurrence. Metastatic pheochromocytoma still remains a therapeutic challenge.