$Unique_ID{BRK04080} $Pretitle{} $Title{Pancreatic Islet-Cell Tumor} $Subject{Pancreatic Islet-Cell Tumor Endocrine Tumor Encephalopathy Hypoglycemic Multiple Endocrine Adenomatosis Zollinger-Ellison Syndrome Hypoglycemia} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 716: Pancreatic Islet-Cell Tumor ** IMPORTANT ** It is possible that the main title of the article (Pancreatic Islet-Cell Tumor) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Endocrine Tumor Encephalopathy, Hypoglycemic Multiple Endocrine Adenomatosis Information on the following diseases can be found in the Related Disorders section of this report: Zollinger-Ellison Syndrome Hypoglycemia General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Pancreatic-islet cell tumors appear in one of two forms. They may be non functioning 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 which 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. Symptoms Symptoms of pancreatic islet-cell tumor vary with the type of hormone- producing tumor that occurs. Generally, symptoms include periodic attacks of pain in the abdomen which increase in severity and frequency. Insulin- secreting tumors may result in abnormally low blood sugar (hypoglycemia), and may produce such symptoms as headache, visual disturbances, confusion, weakness, sweating, uncontrollable tremors or quivering, loss of muscular coordination (ataxia), personality changes, palpitations, convulsions or coma. Symptoms of severe hypoglycemia may be confused with those of many neurologic or psychiatric disorders. Pancreatic islet-cell tumors which produce the hormone gastrin commonly result in multiple ulcers. Approximately 50% of these tumors are benign and their growth and spread are slow. Symptoms of ulcers may include gastrointestinal bleeding, stomach pain and diarrhea. Zollinger-Ellison Syndrome is characterized by multiple ulcers which may be caused by gastrin secreting islet-cell tumors of the pancreas. These ulcers commonly bleed, cause stomach pain and diarrhea. (For more information on this disorder, choose "Zollinger-Ellison" as your search term in the Rare Disease Database). Certain pancreatic islet-cell tumors which secret the hormone vasoactive intestinal polypeptide (VIP) are responsible for such symptoms as severe watery diarrhea, dehydration, intermittent vomiting, weight loss, weakness, decreased potassium in the blood (hypokalemia), excess calcium in the blood (hypercalcemia), reduced or absent gastric secretion and degenerative changes in the spine. This disorder is called Verner-Morrison Syndrome or Pancreatic Cholera. Causes The exact cause of pancreatic islet-cell tumors is not known. However scientists believe certain forms of this disorder may be inherited. Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother. Verner-Morrison syndrome and a particular form of Zollinger-Ellison syndrome are inherited as autosomal dominant traits. In dominant disorders a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is fifty percent for each pregnancy regardless of the sex of the resulting child. Affected Population Pancreatic islet-cell tumors occur most often in males between the ages of 30 and 60. Related Disorders Symptoms of the following disorder can be similar to those of Pancreatic Isleta-Cell Tumors. Comparisons may be useful for a differential diagnosis: Hypoglycemia is characterized by abnormally low blood sugar, and may be caused by excess insulin in the body. Symptoms of hypoglycemia may include faintness, weakness, jitteriness, profuse perspiration, excessive hunger and nervousness. Islet-cell tumors of the pancreas may be one source of the excess insulin, although hypoglycemia often occurs for unknown reasons. (For more information on this disorder, choose "Hypoglycemia" as your search term in the Rare Disease Database). Therapies: Standard Small benign tumors at or near the surface of the pancreas can usually be surgically removed. If the tumor is large or deep into the body or tail of the pancreas, a portion of the pancreas may be removed. Total removal of the pancreas (pancreatectomy) is reserved only for large malignant tumors of the pancreas. Surgical cure rates are very high for small benign tumors. The drugs Diazoxide and Streptozocin (for insulinoma) are frequently administered to control symptoms or reduce the size of the tumor. Gastrin secreting pancreatic islet-cell tumors (Zollinger-Ellison Syndrome) may be treated with the drug Cimetidine which greatly reduces gastric acid output and promotes healing and alleviates symptoms. Surgical removal of the tumor is possible in approximately 20% of all patients. Streptozocin is also used to reduce diarrhea and tumor mass. The drug Sandostatin has been found to be effective in controlling diarrhea and suppressing secretion of the hormones gastrin, insulin, glucagon and VIP by islet-cell tumors. Pancreatic cholera (Verner-Morrison Syndrome) must be treated by replacing fluids and electrolytes lost in numerous watery stools. The tumor may be surgically removed in approximately 50% of the cases. Streptozocin may also be administered to reduce diarrhea and tumor mass. Some individuals may respond to a corticosteroid drug such as Prednisone. Sandostatin may be administered for severe diarrhea and to reduce elevated VIP levels produced by islet-cell tumors. Therapies: Investigational This disease entry is based upon medical information available through July 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Pancreatic Islet-Cell Tumors, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 National Digestive Diseases Information Clearinghouse Box NDDIC Bethesda, MD 20892 (301) 468-6344 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 International Tremor Foundation 360 W. Superior St. Chicago, IL 60610 (312) 664-2344 For genetic information and genetic counseling referrals: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 269-271. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 495-499. ISLET CELL CARCINOMAS OF THE PANCREAS: A TWENTY YEAR EXPERIENCE. G.B. Thompson et al; SURGERY, (Dec 1988; 104(6):1011-1017). NONFUNCTIONING ISLET CELL CARCINOMA OF THE PANCREAS. COMPLETE RESPONSE TO CONTINUOUS 5-FLUOROURACIL INFUSION. R. Hansen et al; CANCER, (Jul 1 1988; 62(1):15-17).