Health Library

Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). These tumors, called gastrinomas, produce a large amount of gastrin. Gastrin is a hormone that causes the stomach to produce acid. With too much gastrin, excess acid is produced, causing ulcers in the stomach or small intestine.

Gastric Ulcer

Gastrinomas occur as single tumors or small multiple tumors. Not only can these tumors lead to ulcers, they can also be cancerous (up to 66% malignant) and spread to the nearby lymph nodes or liver. This happens in about one-third to one-half of the cases of Zollinger-Ellison syndrome.

About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). Patients with MEN 1 have additional endocrine tumors in the brain and neck.

Causes

The cause of Zollinger-Ellison syndrome is unclear. It is very rare; fewer than three out of a million people have the syndrome.

Risk Factors

Factors that may increase the chance of MEN 1 include:

Family history
Problems with the endocrine system
Recurrent peptic ulcer disease

Symptoms

Over 90% of people with Zollinger-Ellison syndrome have symptoms typical of a stomach ulcer.

If you experience any of these symptoms, do not assume it is due to Zollinger-Ellison syndrome. These symptoms may be caused by other, less serious health conditions:

Abdominal pain

Nausea or vomiting

Diarrhea (30%)
Ulceration of stomach and small bowel
Heartburn, difficulty swallowing (60%)
Fatigue
Weight loss
Oily stool
GI bleeding with anemia, black stool, or bloody vomit

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. He or she may also refer you to a gastroenterologist, a specialist who deals with gastrointestinal disorders.

Tests may include the following:

Gastrin stimulation tests

Standard test meal
Calcium
Secretin ( a hormone)

Blood tests

Looking for elevated gastrin levels in blood

Imaging
- Abdominal computerized tomography (CT) scan or magnetic resonance imaging (MRI) of the abdomen to show tumors
- Abdominal ultrasound
Upper gastrointestinal endoscopy
- A thin instrument with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers

Treatment

Talk with your doctor about the best treatment plan for you. Treatment options include:

If there is only one tumor and it is not cancerous, a surgical removal may be attempted.

Proton pump inhibitors are medications that reduce acid by blocking the ‘pumps’ in acid-secreting cells. Examples include Nexium, Prevacid, Protonix, and Prilosec.
Histamine blockers are medications to reduce the amount of hydrochloric acid released by the stomach. This will relieve pain and allow the ulcers to heal.
Chemotherapy such as streptozocin is used in those with malignant form. with or without evidence of cancer spread.
Control tumor growth with the use of somatostatin analogs (eg, octreotide).

Prevention

There are no know prevention steps since the cause is unclear.

American Gastroenterological Association

http://www.gastro.org/

National Digestive Diseases Information Clearinghouse

http://digestive.niddk.nih.gov/

The Canadian Association of Gastroenterology

http://www.cag-acg.org/

Health Canada

http://www.hc-sc.gc.ca/index_e.html/

Berna MJ, Hoffmann KM, Long SH, et al. Serum gastrin in Zollinger-Ellison syndrome: II. Prospective study of gastrin provocative testing in 293 patients from the National institutes of Health and comparison with 537 cases from the literature, evaluation of diagnostic criteria, proposal of new criteria, and correlations with clinical and tumoral features. Medicine. 2006;85:331-64.

Campana D, Piscitelli L, Mazzotta E, et al. Zollinger-Ellison syndrome: diagnosis and therapy. Minerva Med. 2005;96:167-206.

DynaMed Editorial Team. Zollinger-Ellison syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated October 18, 2010. Accessed November 11, 2010.

Mayo Clinic. Zollinger-Ellison syndrome. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/zollinger-ellison-syndrome/DS00461. Updated July 24, 2010. Accessed November 11, 2010.

Norton JA, Fraker DL, Alexander HR, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999;341:635-644.

Norton JA, Jensen RT. Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison Syndrome. Ann Surg. 2004;240:757-773.

Pellicano R, De Angelis C, Resegotti A, Rizzetto M. Zollinger-Ellison syndrome in 2006: concepts from a clinical point of view. Panminerva Med. 2006;48:33-40.

Tomassetti P, Salomone T, Migliori M, et al. Optimal treatment of Zollinger-Ellison syndrome and related conditions in elderly patients. Drgus Aging. 2003;20:1019-1034.

Last reviewed March 2013 by Daus Mahnke, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.