Zollinger Ellison Syndrome / Disease

Diseases & Conditions

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Zollinger Ellison Syndrome

Synonyms of Zollinger Ellison Syndrome

Gastrinoma
Pancreatic Ulcerogenic Tumor Syndrome
ZES
Z-E Syndrome

Disorder Subdivisions

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.

Organizations related to Zollinger Ellison Syndrome

Canadian Multiple Endocrine Neoplasm Society, Inc.
Box 100
Saskatchewan None SOM 1XO
Phone #: 306-892-2080
800 #: --
e-mail: [email protected]
Home page: http://www.mensociety.com
NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Endocrine Diseases Metabolic Diseases Branch
Bethesda MD 20892-3570
Phone #: 301-654-3810
800 #: --
e-mail: [email protected]
Home page: http://www.niddk.nih.gov

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html