Screening for pheochromocytoma in the MEN 2 syndrome

Twenty-two patients with the multiple endocrine neoplasia type 2 (MEN 2) syndrome were screened for pheochromocytoma since it is a major cause of morbidity in MEN 2 families. Clinical symptoms, biochemical parameters, ultrasound, computed tomography or magnetic resonance imaging, and meta-iodo-benzylguanidine (MIBG) scintigraphy were evaluated for detection of adrenal tumors. Clinical symptoms and plasma or urine catecholamines appeared to be nonspecific, whereas MIBG scintigraphy was highly specific and the most sensitive parameter. Patients older than age 30 should be scintigraphically screened at least once despite the radiation exposure. Demonstration of only slight uptake is not an indication for surgery but rather for careful follow-up.