Pancreatic Islet Cell Tumor

National Organization for Rare Disorders, Inc.

It is possible that the main title of the report Pancreatic Islet Cell Tumor is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


  • Encephalopathy, Hypoglycemic
  • Multiple Endocrine Adenomatosis

Disorder Subdivisions

  • None

General Discussion

Pancreatic-islet cell tumors appear in one of two forms. They may be nonfunctioning or functioning tumors. Nonfunctioning tumors may cause obstruction in the shortest part of the small intestine (duodenum) or in the biliary tract, which connects the liver to the duodenum and includes the gall bladder. These nonfunctioning tumors may erode and bleed into the stomach and/or the intestines, or they may cause an abdominal mass.

Functioning tumors secrete excessive amounts of hormones, which may lead to various syndromes including low blood sugar (hypoglycemia), multiple bleeding ulcers (Zollinger-Ellison Syndrome), pancreatic cholera (Verner-Morrison Syndrome), carcinoid syndrome or diabetes.

Islet cells are small, isolated masses of cells that make up the Islet of Langerhans in the pancreas. When functioning normally, they secrete the protein hormones insulin and glucagon. Tumors composed of irregular islet cells may occur alone or in a group of many tumors. Approximately 90% of islet-cell tumors are noncancerous (benign). They usually range in size from 0.5 to 2 cm in diameter.


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Genetic and Rare Diseases (GARD) Information Center
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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

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