Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Zollinger-Ellison Syndrome

  • Wen-Yih LiangEmail author
  • Gregory Y. Lauwers
Reference work entry


Zollinger-Ellison syndrome (ZES) is a complex clinicopathologic condition that develops secondary to autonomous production of gastrin and is characterized by massive acid secretion and severe peptic ulcer disease.

Clinical Features


The autonomous hypergastrinemia leads to persistent and massive secretion of acid and pepsin, giving rise to multiple refractory peptic ulcers. Abdominal pain, primarily related to the ulcers, is observed in the majority of patients and frequently is accompanied by weight loss. Severe esophagitis and symptoms of gastroesophageal reflux disease, as well as duodenojejunitis and duodenal ulcers, are common findings. In addition, diarrhea is observed in 30–70% of patients, with malabsorption and steatorrhea resulting from concentrated acid entering the small bowel. The presenting symptoms are similar in the 25% of patients with Zollinger-Ellison syndrome who have MEN-1 syndrome, although they may be present at a younger age and more...

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References and Further Reading

  1. Berna, M. J., et al. (2008). A prospective study of gastric carcinoids and enterochromaffin-like cell changes in multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: Identification of risk factors. The Journal of Clinical Endocrinology and Metabolism, 93(5), 1582–1591.CrossRefGoogle Scholar
  2. Ellison, E. C., et al. (2009). The Zollinger-Ellison syndrome: A comprehensive review of historical, scientific, and clinical considerations. Current Problems in Surgery, 46(1), 13–106.CrossRefGoogle Scholar
  3. Jensen, R. T., & Norton, J. A. (2010). Chapter 32: Endocrine tumors of the pancreas and gastrointestinal tract. In Sleisenger and Fordtran’s gastrointestinal and liver disease (9th ed., Vol. 1, pp. 491–522). Philadelphia: Saunders Elsevier.CrossRefGoogle Scholar
  4. Metz, D. C., et al. (2001). Replacement of oral proton pump inhibitors with intravenous pantoprazole to effectively control gastric acid hypersecretion in patients with Zollinger-Ellison syndrome. The American Journal of Gastroenterology, 96(12), 3274–3280.CrossRefGoogle Scholar
  5. Norton, J. A., et al. (2007). Role of surgery in Zollinger-Ellison syndrome. Journal of the American College of Surgery, 205(4 Suppl), S34–S37.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Department of PathologyTaipei Veterans General HospitalTaipeiTaiwan
  2. 2.Department of PathologyMassachusetts General Hospital, Harvard Medical SchoolBostonUSA