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Zollinger-Ellison Syndrome

Zollinger-Ellison syndrome (ZES) is a rare digestive disorder. If you have ZES, you likely have one or more tumors in your duodenum (the first part of the small intestine), the pancreas, or both organs. These tumors, called gastrinomas, release the hormone gastrin. This causes the stomach to release too much gastric acid, used to break down food. The extra acid can cause painful peptic ulcers inside the lining of your stomach and intestine. While gastrinoma tumors do cause health problems, they are typically benign (noncancerous) tumors.

Symptoms

The symptoms of ZES are similar to those of other ulcers. They include:

  • Nausea

  • Vomiting

  • Weight loss

  • Diarrhea

  • Abdominal pain, sometimes burning in nature

  • Severe GERD (gastroesophageal reflux disease)

Who’s at risk

Just about anyone can develop Zollinger-Ellison syndrome. However, some people with the condition may have a contributing genetic disorder known as multiple endocrine neoplasia type 1 (MEN1). If you have MEN1, you have a 20 to 60 percent chance of developing ZES. Children of adults with MEN1 are also at an increased risk of getting the disease. Zollinger-Ellison syndrome is more common in men, often those 30 to 50 years old.

Diagnosis

If you have the symptoms associated with ulcers or Zollinger-Ellison syndrome, your health care provider may measure your stomach acid levels. He or she may also give you a blood test to measure your level of the hormone gastrin, to see whether your body produces too much. Your doctor may order imaging tests to look for the tumors that indicate ZES.

Treatment

In some cases of Zollinger-Ellison syndrome, you can take drugs called proton pump inhibitors to manage the extra stomach acid that your stomach produces. These drugs help to stop ulcers from developing. They include esomeprazole (Nexium), lansoprazole (Prevacid), and omeprazole (Prilosec). Sometimes, you may need surgery to remove tumors that have developed in your digestive tract. In the most severe situations, including when tumors have spread to others parts of your body and can’t all be removed, your doctor may give you chemotherapy to destroy them.

Complications

In about 75 percent of people with Zollinger-Ellison Syndrome, tumors grow slowly and do not spread quickly. If you can manage the ulcers, you can enjoy good quality of life. The 10-year survival rate is 95 percent. In about 25 percent of people with Zollinger-Ellison syndrome, however, the tumors are considered cancerous. They spread quickly to other areas of your body, such as the liver and bones. In these instances, the 10-year survival rate drops to about 30 percent.

When to call the doctor

Zollinger-Ellison syndrome is a potentially serious disease with severe complications, if left untreated. If any of the symptoms mentioned above persist for more than a few days, see your health care provider for a diagnosis.