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Zollinger Ellison syndrome

Abstract

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NORD is very grateful to E. Christopher Ellison, MD, Associate Vice-President Health Sciences and Vice-Dean for Clinical Affairs; Chair, The Ohio State University Physicians Board; Robert M.Zollinger Professor and Chair Department of Surgery, The Ohio State University Medical Center, for assistance in the preparation of this report.

Synonyms of Zollinger Ellison syndrome

  • Gastrinoma
  • Pancreatic Ulcerogenic Tumor Syndrome
  • ZES
  • Z-E Syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Zollinger-Ellison syndrome (ZES) is characterized by the development of a tumor (gastrinoma) or tumors that secrete excessive levels of gastrin, a hormone that stimulates production of acid by the stomach. Many affected individuals develop multiple gastrinomas, which are thought to have the potential to be cancerous (malignant). In most cases, the tumors arise within the pancreas and/or the upper region of the small intestine (duodenum).

Due to excessive acid production (gastric acid hypersecretion), individuals with ZES may develop peptic ulcers of the stomach, the duodenum, and/or other regions of the digestive tract. Peptic ulcers are sores or raw areas within the digestive tract where the lining has been eroded by stomach acid and digestive juices. Symptoms and findings associated with ZES may include mild to severe abdominal pain; diarrhea; increased amounts of fat in the stools (steatorrhea); and/or other abnormalities.

In most affected individuals, ZES appears to develop randomly (sporadically) for unknown reasons. In approximately 25 percent of cases, ZES occurs in association with a genetic syndrome known as multiple endocrine neoplasia type 1 (MEN-1). All of the tumors are considered to have malignant potential. Prognosis is related to tumor size and the presence of distant metastases.

Zollinger Ellison syndrome Resources

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