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.
D007516:Adenoma, Islet Cell / Q000601:surgery*; D002763:Cholecystectomy; D005743:Gastrectomy; D005744:Gastric Acid / Q000378:metabolism; D006801:Humans; D007340:Insulinoma / Q000601:surgery*; D007813:Laparotomy; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000601:surgery*; D010190:Pancreatic Neoplasms / Q000601:surgery*; D010280:Parathyroid Glands / Q000601:surgery; D012086:Reoperation; D015043:Zollinger-Ellison Syndrome / Q000188:drug therapy / Q000378:metabolism / Q000601:surgery*