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Zollinger–Ellison Syndrome

Definition

Disorder characterized by hypergastrinemia and increased acid secretion. May be part of MEN syndrome.

Clinical Features

  • Sometimes:
    • part of multiple endocrine neoplasia
    • associated multicentric carcinoid tumors:

Pathogenesis

  • Poorly understood
  • Hyperplasia of parietal and ECL cells presumably result from gastrin stimulation1

Histopathology

  • Hyperplasia:
    • primarily affecting secretory portion of fundic gland(Figs 1 and 2
      Medium-power view of gastric mucosa in a patient with Zollinger–Ellison syndrome. Note the large increase in the number of parietal cells.

      Fig. 1: Medium-power view of gastric mucosa in a patient with Zollinger–Ellison syndrome. Note the large increase in the number of parietal cells.

      High-power view of gastric mucosa in a patient with Zollinger–Ellison syndrome. Note the large increase in the number of parietal cells.

      Fig. 2: High-power view of gastric mucosa in a patient with Zollinger–Ellison syndrome. Note the large increase in the number of parietal cells.

      )
    • mainly involves parietal cells
    • may be increase in number of ECL cells
  • If associated multicentric carcinoid tumors:
    • mainly:
      • in fundus
      • composed of ECL cells
  • May be:
    • fundic gland polyps
    • intramucosal cysts2

Diagnosis

  • May be gastric changes radiographically and grossly similar to those of Menétrier's disease
  • Probably clinicopathologic variations:
    • some lesions have similar morphologic features but without hypergastrinemia:
      • some with:
        • protein loss
        • gastric adenocarcinoma3–5

Genetics

TypeNameComment

References

1 Delle Fave G, Marignani M, Corleto VD, Angeletti S, D'Ambra G, Ferraro G, et al. Progression of gastric enterochromaffin-like cells growth in Zollinger-Ellison syndrome and atrophic body gastritis patients. Dig Liver Dis. 2002;34:270–278.

2 Aprile MR, Azzoni C, Gibril F, Jensen RT, Bordi C. Intramucosal cysts in gastric body of patients with Zollinger-Ellison syndrome. Hum Pathol. 2000;31:140–148.

3 Komorowski RA, Caya JG. Hyperplastic gastropathy. Clinicopathologic correlation. Am J Surg Pathol. 1991;15:577–585.

4 Solcia E, Capella C, Buffaetal R. Pathology of the Zollinger-Ellison syndrome. Progress in surgical pathology. 1980;1:119–134In Fenoglio CM, Wolff M (eds).

5 Stamm B, Saremaslani P. Coincidence of fundic glandular hyperplasia and carcinoma of the stomach. Cancer. 1989;63:354–359.

Last updated: 28 Nov 2006

Zollinger–Ellison Syndrome

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