Zollinger-Ellison syndrome is a complex condition in which one or more tumors form in your pancreas, the upper part of your small intestine (duodenum) or the lymph nodes adjacent to your pancreas. 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, also called ZES, is rare. The disease may occur at any time in life, but people are usually diagnosed between ages 20 and 50. It's slightly more common in men.
Treatment for Zollinger-Ellison syndrome most often consists of medications to reduce stomach acid and heal the ulcers. Surgery to remove the tumors may be an option for some people with Zollinger-Ellison syndrome.
Zollinger-Ellison syndrome causes signs and symptoms similar to those of a peptic ulcer. These 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 is located 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. The duodenum, the upper part of the small intestine, begins at the lower end of your stomach. It's in the duodenum, where digestive juices from the pancreas, liver and gallbladder mix, that digestion reaches its peak.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete very 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.
In addition to 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.
Association with MEN I
The pancreas is a large organ that lies horizontally in your upper abdomen behind your stomach. ...
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 gastroenterologist to diagnose and treat Zollinger-Ellison syndrome. You may also be referred to an oncologist, a doctor who specializes in treating cancer.
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared. Here's some information to help you get ready, and what to expect from your doctor.
What you can do
Preparing a list of questions will help you make the most of the limited time you have with your doctor. For Zollinger-Ellison syndrome, some basic questions to ask your doctor include:
What to expect from your doctor
Tests and diagnosis
Your doctor may review or recommend 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, which are what cause the ulcers, 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: 2010-07-24
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