Insulinoma and gastrinoma in Wermer's disease (MEN I)

The association of gastrin- and insulin-producing tumors of the pancreas is rare. In the 30 years' experience of Zollinger and others, of 40 patients with gastrinoma none had insulin-producing tumors. In contrast to patients with Zollinger-Ellison syndrome, of whom 15% to 26% are classified as having multiple endocrine neoplasias type I (MEN I), only 3% to 4% of patients with insulinomas have other endocrine neoplasms. Insulinomas in patients with MEN I are usually single tumors that usually can be cured with enucleation of the tumor. In contradistinction, gastrinomas in patients with MEN I are diffuse in nature and resection only rarely can be accomplished. Long-term management of gastric hypersecretion is best accomplished by H2-receptor antagonists. If the patient does not respond to H2-receptor antagonists or is unwilling to take the drug indefinitely, he or she will be a candidate for total gastrectomy.