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Zollinger-Ellison syndrome

Zollinger-Ellison syndrome is a disorder where increased levels of the hormone gastrin are produced, causing the stomach to produce excess hydrochloric acid. Often, the cause is a tumour of the pancreas producing the hormone gastrin. As these tumors are benign, they are only removed if the disease cannot be controlled with medication. more...

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Zollinger-Ellison syndrome
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Causes

Zollinger-Ellison syndrome is caused by tumors usually found in the head of the pancreas and the upper small bowel. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid.

Gastrin works on stomach parietal cells causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus, there is an increase in the number of acid secreting cells and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach and duodenum. High acid levels lead to multiple ulcers in the stomach and small bowel.

Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel.

Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.

Symptoms

  • pain
  • vomiting blood (occasional)
  • difficulty in eating

Therapy

Proton pump inhibitors and H2 blockers are used to slow down acid secretion. If possible the tumours should be surgically removed, or treated with chemotherapy.

History

The disease entity was first described in 1955 by its namesakes: Zollinger RM, Ellison EH. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg 1955;142:709-23. PMID 13259432.

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Zollinger-Ellison Syndrome
From Pamphlet by: National Institute of Diabetes & Digestive & Kidney Diseases, 3/17/98

Zollinger-Ellison syndrome is a rare disorder that causes tumors in the pancreas and duodenum and ulcers in the stomach and duodenum. The pancreas is a gland located behind the stomach. It produces enzymes that break down fat, protein, and carbohydrates from food and hormones like insulin that break down sugar. The duodenum is the top part of the small intestine.

The tumors are cancerous in 50 percent of cases. They secrete a substance called gastrin that causes the stomach to produce too much acid, which in turn causes the stomach and duodenal ulcers (peptic ulcers). The ulcers caused by Zollinger-Ellison syndrome are more painful and less responsive to treatment than ordinary peptic ulcers. What causes people with Zollinger-Ellison syndrome to develop tumors is unknown, but the cause may be an abnormal tumor suppressor gene.

Zollinger-Ellison syndrome usually occurs in people between ages 30 and 60. Symptoms include signs of peptic ulcers: gnawing, burning pain in the abdomen; nausea; vomiting; fatigue; weakness; and weight loss. Diarrhea is also a symptom. Physicians diagnose Zollinger-Ellison syndrome through blood tests to measure levels of gastrin. They may check for ulcers by taking x-rays of the stomach and duodenum or by doing an endoscopy, which involves looking at the lining of these organs through a lighted tube.

Medications used to reduce stomach acid include cimetidine, ranitidine, famotidine, and omeprazole. Surgery to treat peptic ulcers or to remove tumors in the pancreas or duodenum are other treatment options. In serious cases, surgery to remove the entire stomach may be necessary.

More information is available from

Additional Information on Zollinger-Ellison Syndrome

The National Digestive Diseases Information Clearinghouse collects resource information on digestive diseases for the Combined Health Information Database (CHID). CHID is a database produced by health-related agencies of the Federal Government. This database provides titles, abstracts, and availability information for health information and health education resources.

To provide you with the most up-to-date resources, information specialists at the clearinghouse created an automatic search of CHID. To obtain this information you may view the results of the automatic search on Zollinger-Ellison Syndrome.

Or, if you wish to perform your own search of the database, you may access the CHID Online web site and search CHID yourself.

National Digestive Diseases Information Clearinghouse

The National Digestive Diseases Information Clearinghouse (NDDIC) is a service of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). The NIDDK is part of the National Institutes of Health under the U.S. Department of Health and Human Services. Established in 1980, the clearinghouse provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. NDDIC answers inquiries; develops, reviews, and distributes publications; and works closely with professional and patient organizations and Government agencies to coordinate resources about digestive diseases.

Publications produced by the clearinghouse are reviewed carefully for scientific accuracy, content, and readability.

This e-text is not copyrighted. The clearinghouse encourages users of this e-pub to duplicate and distribute as many copies as desired.

e-text posted: 17 March 1998

COPYRIGHT 1998 National Institute of Diabetes & Digestive & Kidney Diseases
COPYRIGHT 2004 Gale Group

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