[Pheochromocytoma - still a challenge]

Pheochromocytomas are rare, mostly benign catecholamine-producing tumors arising from the chromaffin cells of the adrenal medulla or in the paraganglia. Clinical presentation is highly variable but typically with hypertension, severe headaches, palpitations and sweating. Biochemical testing by 24 h urinary fractioned metanephrines or catecholamines and plasma free metanephrines as the most sensitive screening approach, confirms the catecholamine excess. Computed tomography scan and magnetic resonance imaging of the adrenal glands and abdomen as well as functional imaging with (123)Iod-MIBG scintigraphy and (18)F-dopa positron emission tomography are used for tumor localization. Because approximately a quarter of tumors develop secondary to germ-line mutations, screening for genetic alterations is important. The therapy of choice is the endoscopic adrenal sparing surgery following preoperative alpha-blockade. Regular follow-up remains essential due to possible recurrence and malignancy.