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 Copyright © Nucleus Medical Media, Inc.

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    TOP

Factors that may increase the chance of MEN 1 include:

• Family history
• Problems with the endocrine system
• Recurrent peptic ulcer disease

Symptoms    TOP

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    TOP

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    TOP

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

Prevention    TOP

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