Zollinger-Ellison syndrome is a complex condition in which one or more tumors form in your pancreas or the upper part of your small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes your stomach to produce too much acid. The excess acid, in turn, leads to peptic ulcers.
Zollinger-Ellison syndrome (ZES) is rare. The disease may occur at any time in life, but people are usually diagnosed between ages 30 and 50. Medications to reduce stomach acid and heal the ulcers is the usual treatment for Zollinger-Ellison syndrome.
Signs and symptoms of Zollinger-Ellison syndrome may include:
When to see a doctor
Tell your doctor if you've used over-the-counter acid-reducing medications such as omeprazole (Prilosec), cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time. These medications may mask your symptoms, which could delay your diagnosis. If you have Zollinger-Ellison syndrome, early detection and treatment are important.
The exact cause of Zollinger-Ellison syndrome remains unknown. But the sequence of events that occurs in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas, duodenum or the lymph nodes adjacent to your pancreas.
Your pancreas sits behind and below your stomach. It produces enzymes that are essential to digesting food. The pancreas also produces several hormones, including gastrin, a hormone that controls stomach acid production. Digestive juices from the pancreas, liver and gallbladder mix in the duodenum, the part of the small intestine next to your stomach. This is where digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of gastrin, which in turn causes the stomach to produce far too much acid. The excessive acid then leads to peptic ulcers and sometimes to diarrhea.
Besides causing excess acid production, the tumors may be cancerous (malignant). The tumors themselves grow slowly, but the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
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Preparing for your appointment
Although your symptoms may prompt you to visit your family doctor or a general practitioner, you'll likely be referred to a doctor who specializes in diseases of the digestive system (gastroenterologist) to diagnose and treat Zollinger-Ellison syndrome. You also may be referred to an oncologist, a doctor who specializes in treating cancer.
Here's some information to help you get ready for your appointment and know what to expect from your doctor.
What you can do
Questions to ask your doctor
What to expect from your doctor
Tests and diagnosis
Your doctor will base a diagnosis on the following:
Treatments and drugs
In treating Zollinger-Ellison syndrome, doctors treat the tumors as well as the ulcers. If your doctor can remove the tumors, then ulcer treatment may no longer be needed.
Treatment of tumors
In some cases, doctors advise other treatments to control tumor growth, including:
More radical surgical approaches, such as severing the nerves that promote acid secretion or removing the entire stomach, aren't generally done today because medications are usually successful in controlling acid production and ulcers.
Treatment of excess acid
Your doctor may also suggest one of several operations to treat peptic ulcers, such as surgery to:
Last Updated: 2012-10-11
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