Zollinger-Ellison Syndrome

(Z-E Syndrome; Gastrinoma)

Definition

Zollinger-Ellison syndrome is a rare disorder that arises from tumors and causes ulcers in the digestive system. One or more tumors form in the pancreas or duodenum (the upper part of the small intestine). Not only can these tumors lead to ulcers, they can also be cancerous, and spread to the nearby lymph nodes or liver.About one-quarter of people with Zollinger-Ellison syndrome have a genetic disorder called multiple endocrine neoplasia type 1 (MEN 1). People with MEN 1 may have additional endocrine tumors in the brain and neck.
Gastric Ulcer
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Causes

Zollinger-Ellison syndrome is caused by gastrin-producing tumors, called gastrinomas. Gastrin is a hormone that stimulates the stomach to produce acid. Excess acid production may lead to ulcers in the stomach or duodenum.

Risk Factors

Factors that may increase your chance of Zollinger-Ellison syndrome include:
  • Your or family members with MEN 1
  • History of endocrine disorders
  • Recurrent peptic ulcers

Symptoms

In many people, Zollinger-Ellison syndrome causes symptoms similar to an ulcer or gastroesophageal reflux disease (GERD). Symptoms may include:
  • Abdominal pain
  • Heartburn
  • Nausea or vomiting
  • Fatigue
  • Diarrhea
  • Weight loss
  • Blood in the vomit or stool

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done.Tests may include:
  • Blood tests—specifically to look for elevated levels of the hormone gastrin or evidence of MEN 1
  • Upper gastrointestinal endoscopy—a flexible tube with a light and camera is inserted down the throat and into the stomach and intestine to look for ulcers
Imaging tests may help your doctor to detect and localize the tumors. These tests may include:

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