$Unique_ID{BRK04348} $Pretitle{} $Title{Zollinger-Ellison Syndrome} $Subject{Zollinger-Ellison Syndrome Pancreatic Ulcerogenic Tumor Syndrome Multiple Endocrine Neoplasia Type I Partial Multiple Endocrine Adenomatosis Z-E Syndrome Gastrinoma Cushing Syndrome Duodenal Ulcers } $Volume{} $Log{} Copyright (C) 1987, 1988, 1990 National Organization for Rare Disorders, Inc. 360: Zollinger-Ellison Syndrome ** IMPORTANT ** It is possible the main title of the article (Zollinger-Ellison Syndrome) is not the name you expected. Please check the SYNONYMS listing to find the alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Pancreatic Ulcerogenic Tumor Syndrome Multiple Endocrine Neoplasia, Type I Partial Multiple Endocrine Adenomatosis Z-E Syndrome Gastrinoma Information on the following diseases can be found in the Related Disorders section of this report: Cushing Syndrome Duodenal Ulcers 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. Zollinger-Ellison Syndrome is an unusual ulcerative condition characterized by small tumors (usually of the pancreas) which secrete a hormone that produces excess amounts of stomach (gastric) juices. These tumors can also appear in the lower stomach wall, spleen or lymph nodes close to the stomach. Large amounts of gastric acid can be found in lower stomach areas where ulcers can form. Ulcers can appear suddenly even in areas where they are rarely found, may persist following treatment, and can be accompanied by diarrhea. Prompt medical treatment of these ulcers is necessary to prevent complications such as bleeding and perforation. Symptoms Tumors which characterize Zollinger-Ellison Syndrome secrete a hormone which produces excess amounts of stomach acid which can cause ulcers in the stomach, duodenum and jejunum, and in the esophagus due to a backward flow of gastric acid from the stomach. Pain from these persistent ulcers may be severe. Diarrhea and excretion of fat in the feces (steatorrhea) commonly occurs. Ulcers may persist for years despite medical or surgical treatment. This can result in a decrease of potassium levels in the blood. Complications, including holes in the organ walls where ulcers occur (perforations), bleeding or obstruction of the stomach where it empties into the intestines, may be serious and require prompt treatment. Causes Two common forms of Zollinger-Ellison Syndrome were identified in 1982. One form begins sporadically, usually during late adulthood, and is usually malignant. The other form is inherited as an autosomal dominant trait and occurs as a symptom of Multiple Endocrine Adenomatosis. (Human traits including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.) Other forms of Zollinger- Ellison Syndrome may also exist. The mechanism that causes the tumors to grow is unknown. Affected Population Zollinger-Ellison Syndrome can begin in childhood, but it is usually diagnosed between twenty to seventy years of age. This disorder seems to affect males and females in equal numbers. Related Disorders Zollinger-Ellison Syndrome may be associated with endocrine disorders such as increased activity of the parathyroid glands (hyperparathyroidism) and benign tissue cell growth of the adrenal or pituitary glands (adenomas). Cushing Syndrome consists of a group of symptoms attributable to an excess of cortisol and other hormones from the cortex of the adrenal gland. It is usually caused by hormone secreting tumors of the adrenal or pituitary glands; sometimes a hyperfunctioning pituitary gland can be the cause. Hormone secreting tumors may also develop in other organs. Cushing syndrome occurs more frequently in females than in males, particularly women in their thirties following a pregnancy. The prognosis is good if the causative tumors can be removed and/or drug therapy suppresses production of the hormone. (For more information, choose "Cushing" as your search term in the Rare Disease Database.) Duodenal ulcers are very common. A possible genetic predisposition to the disorder is possible. Steroid drugs can be one cause of these ulcers. Other possible causes include emotional stress, complications of cirrhosis of the liver, chronic pancreatitis, cystic fibrosis, or pulmonary emphysema. Approximately ten percent of the United States population may be affected by duodenal ulcers at some time. The highest incidence occurs in Americans between twenty and fifty years of age, although duodenal ulcers can occur in children. The prognosis is generally favorable with standard treatments. However, without treatment duodenal ulcers can be serious due to possible bleeding (hemorrhage), low red blood cell count (anemia), or formation of small holes in the wall of organs where the ulcers occur (perforations). Therapies: Standard Patients with Zollinger-Ellison Syndrome are treated to control or reduce gastric acid production. In the past the most serious cases were treated by removal of the stomach (gastrectomy). This has been replaced (excluding the most serious cases) by use of currently available antacids and drugs such as cimetidine or ranitidine. These drugs (with or without an anticholinergic agent) control excess gastric acid in most patients. Many patients with Z-E Syndrome require higher doses than those used routinely to treat ulcers. Imaging procedures now in use to locate the tumors are improving the success rate of treatment. These procedures include ultrasound, CT scan, and selective angiograms. Abdominal surgery to remove tumors may be indicated and anti-tumor chemotherapy may be of benefit in some cases. Genetic counseling can be useful to families of patients with Z-E Syndrome. In March 1989 the FDA approved a new drug, Losec, for the treatment of Zollinger-Ellison Syndrome and other serious gastric disorders. Losec is manufactured by Merck & Company. Therapies: Investigational New tests are being developed for the diagnosis of Zollinger-Ellison Syndrome such as transhepatic pancreatic vein catheterization supplemented by determination of local hormone gradients. This may permit preoperative location of even the smallest tumors. At present, the success rate of tumor surgery is approximately twenty percent. Surgery on the tenth cranial (vagus) nerve is being tried experimentally. Severing this nerve interrupts impulses to the acid-secreting glands of the stomach. Those who require very high doses of drugs such as cimetidine or ranitidine may be candidates for this surgery which is recommended only for the most serious cases. Newer more potent drugs being developed for treatment of Z-E Syndrome include omeprazole. This drug is still experimental in the United States. This disease entry is based upon medical information available through January 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 Zollinger-Ellison Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Digestive Diseases Information Clearinghouse Box NDIC Bethesda, MD 20892 (301) 468-2162 For information on genetics and genetic counseling referrals, please contact: 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 CURRENT MANAGEMENT OF ZOLLINGER-ELLISON SYNDROME: R.T. Jensen, et. al.; Drugs (August 1986, issue 32(2)). Pp. 188-196. DIAGNOSIS AND CURATIVE THERAPY IN ZOLLINGER-ELLISON SYNDROME: W.H. Hacki; Schweiz Med Wochenschr (April 27, 1985, issue 115(17). Pp. 575-581. ZOLLINGER-ELLISON SYNDROME: H.D. Becker; Wien Klin Wochenschr (February 17, 1984, issue 96(4)). Pp. 138-144.