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Zollinger-Ellison Syndrome

Symptoms - Causes - Diagnosis

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Updated August 07, 2011

What is Zollinger-Ellison Syndrome

Zollinger-Ellison Syndrome (ZES) is a rare disorder that causes one or more tumors to form in the pancreas or the upper part of the small intestine called the duodenum. It can also cause ulcers to develop in the stomach and the duodenum.

The tumors are called gastrinomas, and they secrete a large amount of the hormone gastrin. This then causes an excessive production of stomach acid, which can lead to peptic ulcers.

Zollinger-Ellison syndrome is rare, and though it may occur at any age, people between the ages of 30 and 60 are more likely to develop it. Also, of all the people who suffer with a peptic ulcer, only a tiny percentage of those people will have Zollinger-Ellison.

The tumors are cancerous in 50 percent of the cases. They secrete a hormone called gastrin that causes the stomach to produce too much acid, which in turn causes stomach and duodenal ulcers (peptic ulcers). The ulcers caused by ZES are less responsive to treatment than ordinary peptic ulcers. What causes people with ZES to develop tumors is unknown, but approximately 25 percent of ZES cases are associated with a genetic disorder called multiple endocrine neoplasia.

Symptoms of Zollinger-Ellison Syndrome

  • Gnawing, burning pain in the abdomen
      This pain is usually located in the area between the breastbone and the navel.
  • Sensation of pressure, bloating, or fullness
      This pain usually develops 30 to 90 minutes after a meal, and is often relieved by antacids.
  • Pain or burning sensation in the abdomen that travels up toward the throat
  • Vomiting
      The vomit may contain blood or resemble coffee grounds.
  • Diarrhea
      Stools may be foul smelling.
  • Black, tarry stools
      Blood in the stools will turn them dark red or black, and make them tarry or sticky.
  • Nausea
  • Fatigue
  • Weakness
  • Weight loss

Causes of Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome is caused by a tumor (gastrinoma) or tumors in the pancreas and the upper small bowel (duodenum). These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid. This increase in acidity can lead to the development of peptic ulcers in the stomach and duodenum.

Diagnosing Zollinger-Ellison Syndrome

Blood test.
A blood test is performed to see whether there is an increased gastrin level in the blood. An elevated level of gastrin may indicate tumors in the pancreas or duodenum.

Barium X-ray.
The patient drinks a liquid that contains barium, which will coat the walls of the esophagus, stomach, and duodenum. X-rays are then taken. The doctor will then view the X-rays, looking for signs of ulcers.

Upper endoscopy.
The doctor examines the inside of the esophagus, stomach, and duodenum with an instrument called an endoscope, a thin flexible lighted tube with a lens. The endoscope is inserted through the mouth and down the throat, and into the stomach and duodenum. The doctor can look for ulcers, and can also remove a tissue sample, called a biopsy, for examination in the laboratory to identify if there is the presence of gastrin-producing tumors.

Imaging techniques.
A doctor may use a computerized tomography (CT) scan, a magnetic resonance imaging (MRI) scan, and ultrasound, or a nuclear scan in an effort to pinpoint where tumors may be located.

    A CT scan is a diagnostic test that uses X-rays with aided by computer technology. The X-ray beams are taken from many different angles to create cross-sectional images of the patient's body. Then a computer assembles these images into a three-dimensional picture that can display organs, bones, and tissues in great detail.

    With the MRI scan, magnetic signals are used rather than X-rays to create images of the human body. These images show the differences between types of tissues.

    An ultrasound sends out high frequency sound waves which go into area being examined, and bounce back when they hit an organ. This is processed by a computer, which produces a map of the area being scanned.

    With the nuclear scan, radioactive substances are introduced into the body that permit a gamma camera to detect tumors.

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Part 1: Symptoms - Causes - Diagnosis
Part 2: Complications - Treatment - Prognosis

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  4. Other Digestive Disorders
  5. What is Zollinger Ellison Syndrome

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