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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)

Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type I.

Treatment of the Zollinger-Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type I (MEN I) is controversial. Unlike other patients with ZES who have a single tumor and in whom curative surgical resection is possible and desirable, patients with ZES and MEN I tend to have multiple pancreatic islet cell tumors. Therefore the chances for surgical cure are usually different. A retrospective review of 25 patients with ZES as a manifestation of MEN I seen at our institution between 1960 and 1984, an interval during which histamine type 2 (H2) receptor antagonists were introduced, provided an opportunity to study this problem. Follow-up averaged 6 1/2 years. All patients diagnosed before 1979 (14 patients) underwent surgical exploration. Ten patients (71%) had pancreatic procedures (five enucleation, four partial pancreatectomy, and one enucleation of multiple nodules with subsequent total pancreatectomy). None of these procedures was curative, and all patients required further medical and/or surgical therapy for peptic ulceration. Eighteen patients (72%) underwent gastric surgery. Seven patients had subtotal gastrectomy, five of whom required subsequent total gastrectomy. Thirteen patients ultimately had total gastrectomy. There were no immediate postoperative deaths. All patients with diagnoses since 1979 (11 patients) had H2-antagonist therapy as primary treatment. No significant complications were associated with this approach during a mean treatment period of 26 months. Only one of these patients subsequently has required gastric surgery. The proved efficacy of H2 antagonists, coupled with the recognition of the diffuse nature of the pancreatic disease in these patients, has led to a change in the therapeutic approach over the period reviewed. H2-antagonist therapy should be the initial treatment of choice in patients with MEN I who have ZES.[1]


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