Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the zollinger-ellison syndrome

Miriam Pipeleers-Marichal, Guido Somers, Gerard Willems, Alan Foulis, Clem Imrie, Anne E. Bishop, Julia M. Polak, Walter H. Häcki, Bernhard Stamm, Philipp U. Heitz, Günter Klöppel

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342 Scopus citations


In patients with multiple endocrine neoplasia type 1 (MEN-1), gastrinomas are common and thought to occur predominantly in the pancreas. We describe eight patients with MEN-1 and hypergastrinemia (seven with the Zollinger—Ellison syndrome) in whom we searched for neuroendocrine tumors in the pancreas and duodenum. Tumors were found in the proximal duodenum in all eight patients: solitary tumors (diameter, 6 to 20 mm) in three patients and multiple microtumors (diameter, 2 to 6 mm) in the other five. Paraduodenal lymph-node metastases were detected in four patients. Immunocytochemical analysis revealed the presence of gastrin in all the duodenal tumors and in their lymph-node metastases. In contrast, no immunoreactivity for gastrin was present in the endocrine tumors found in the seven pancreatic specimens available for study, except for one tumor with scattered gastrin-positive cells. In four of the six patients whose duodenal gastrinomas were removed, serum gastrin levels returned to normal; in the other two patients gastrin concentrations decreased toward normal. We conclude that in patients with MEN-1 and the Zollinger—Ellison syndrome, gastrinomas occur in the duodenum, but the tumors may be so small that they escape detection. (N Engl J Med 1990; 322:723–7.).

Original languageEnglish
Pages (from-to)723-727
Number of pages5
JournalNew England Journal of Medicine
Issue number11
StatePublished - 15 Mar 1990
Externally publishedYes


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