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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
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
Meota
Saskatchewan None SOM 1XO
Phone #: 306-892-2080
800 #: --
e-mail: men.society@sasketel.net
Home page: http://www.mensociety.com
- Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
e-mail: ordr@od.nih.gov
Home page: http://rarediseases.info.nih.gov/Default.aspx
- NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Endocrine Diseases Metabolic Diseases Branch
2 Information Way
Bethesda MD 20892-3570
Phone #: 301-654-3810
800 #: --
e-mail: NDDIC@info.niddk.nih.gov
Home page: http://www.niddk.nih.gov
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