Pheochromocytoma in multiple endocrine neoplasia

Three patients with a surgically confirmed and 1 with suspected pheochromocytoma as part of the syndrome of multiple endocrine neoplasia (MEN) type II are presented. Pheochromocytoma and medullary thyroid carcinoma (MTC) as well as other tumors are part of this heritable entity. Three cases were members of a MEN IIa family and 1 patient has suffered from a sporadic form of MEN IIb. Pheochromocytoma was diagnosed by screening procedures (catecholamine screening, computerized tomography, sonography or selective venous catheterization). In 2 of the patients there was no clinical manifestation of the pheochromocytoma. On three occasions MTC was diagnosed first, the thyroid tumor occurred prior to the pheochromocytoma by 7, 6, and 1/2 years. Pheochromocytoma occurred bilaterally in 2 patients, in 1 it is suspected in both adrenals.