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Zollinger-Ellison Syndrome

Definition:
Zollinger-Ellison syndrome is a complex condition in which one or more tumors form in the pancreas or in the upper part of the small intestine (duodenum). These tumors, called gastrinomas, secrete large amounts of the hormone gastrin, which causes excessive production of acid by your stomach. The excess acid, in turn, leads to peptic ulcers.

The ulcers that occur as part of Zollinger-Ellison syndrome are often numerous, tend to occur in unusual areas of your stomach or intestine and are more resistant to treatment than other ulcers are. They are also likely to return after being treated.

Zollinger-Ellison syndrome is rare, affecting approximately one in 1 million people. In the United States, less than 1 percent of duodenal ulcers result from Zollinger-Ellison. The disease may occur at any time, but the average age at diagnosis is 50.

Most often, treatment for Zollinger-Ellison syndrome consists of medications to reduce acid and heal the ulcers. Surgery to remove the tumors may be an option for some people with Zollinger-Ellison.

Causes:
Doctors don't know exactly what causes Zollinger-Ellison syndrome. But the sequence of events in Zollinger-Ellison syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas or duodenum.

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 insulin and glucagon, both of which regulate blood sugar levels, as well as the stomach hormone gastrin, which controls production of stomach acid. The duodenum, the upper part of the small intestine, begins at the lower end of your stomach. In the duodenum, digestive juices from the pancreas, liver and gallbladder mix, and 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 cause 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 — usually to nearby lymph nodes or your liver.

Zollinger-Ellison syndrome may be associated with another disease called multiple endocrine neoplasia, type I (MEN I). People with MEN I have multiple tumors in the endocrine system in addition to pancreatic tumors. They also have tumors in the parathyroid glands and may have tumors in their pituitary glands. About 25 percent of people who have gastrinomas have them as part of MEN I.


When to seek medical advice:
See your doctor if you have a persistent burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea. Don't use over-the-counter acid-blocking medication such as cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time without your doctor's knowledge. These medications may mask your symptoms, which could delay your diagnosis. If you have Zollinger-Ellison syndrome, early detection and treatment are important to prevent the spread of cancer to the lymph nodes or liver.

Symptoms:
See your doctor if you have a persistent burning, aching or gnawing pain in your upper abdomen, especially if you've also been experiencing nausea, vomiting and diarrhea. Don't use over-the-counter acid-blocking medication such as cimetidine (Tagamet), famotidine (Pepcid) or ranitidine (Zantac) for long periods of time without your doctor's knowledge. These medications may mask your symptoms, which could delay your diagnosis. If you have Zollinger-Ellison syndrome, early detection and treatment are important to prevent the spread of cancer to the lymph nodes or liver.

Diagnosis:
Besides reviewing your medical history and asking about your signs and symptoms, your doctor may recommend the following diagnostic procedures:
  • Blood tests. A sample of your blood is analyzed in a laboratory to see whether you have an increased gastrin level in your blood. An elevated gastrin level may indicate tumors in your pancreas or duodenum.
  • Upper gastrointestinal endoscopy. Your doctor inserts a thin, flexible instrument with a light and video camera (endoscope) down your throat and into your stomach and duodenum to look for ulcers. Through the endoscope, your doctor may remove a tissue sample (biopsy) from your duodenum for examination in the laboratory to help detect the presence of gastrin-producing tumors.
  • Imaging techniques. In order to pinpoint the location of tumors, your doctor may use imaging techniques such as ultrasound imaging, a nuclear scan — which uses radioactive tracers to help locate tumors E — computerized tomography (CT) or magnetic resonance imaging (MRI).
  • Endoscopic ultrasound. In this procedure, your doctor examines your stomach and duodenum with an endoscope fitted with an ultrasound probe. The probe allows closer imaging of the digestive tract, making it easier to spot tumors. It's also possible to remove a tissue sample through the endoscope.
Treatment:
In treating Zollinger-Ellison syndrome, doctors focus first on the tumors and then on the ulcers.

Treatment of tumors
An operation to remove the tumors that occur in Zollinger-Ellison requires great skill because the tumors are often small and difficult to locate. If you have just one tumor, your doctor may be able to remove it surgically, but surgery may not be an option if you have multiple tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your doctor still may recommend removing a single large tumor.

In some cases, doctors advise other treatments to control tumor growth, including:
  • Removing as much of a liver tumor as possible (debulking)
  • Attempting to destroy the tumor by cutting off the blood supply (embolization) or by using heat to destroy cancer cells (radio frequency ablation)
  • Injecting drugs into the tumor to relieve cancer symptoms
  • Using chemotherapy to try to slow tumor growth
Treatment of ulcers
Excess acid production can be controlled in almost every person with Zollinger-Ellison syndrome, using one of the following medications:
  • Proton pump inhibitors. These are the most effective medications for decreasing acid production in Zollinger-Ellison syndrome. Proton pump inhibitors are powerful drugs that reduce acid by blocking the action of the tiny "pumps" within acid-secreting cells.
  • Acid blockers. Also called histamine (H-2) blockers, these medications reduce the amount of hydrochloric acid released into your digestive tract. They work by keeping histamine from reaching histamine receptors. Histamine is a substance normally present in your body. When it reacts with histamine receptors, the receptors signal acid-secreting cells in your stomach to release hydrochloric acid. Acid blockers don't work as well as proton pump inhibitors do in treating the ulcers of Zollinger-Ellison syndrome, and most people need high, frequent doses of acid blockers for them to be effective.
Your doctor may also suggest one of several operations to treat peptic ulcers. This may include surgery to:
  • Stop an ulcer from bleeding
  • Relieve an obstruction caused by an ulcer
  • Close up the hole (perforation) that an ulcer has made in the wall of your stomach or duodenum

Your doctor may also suggest severing the nerves that promote acid secretion in your stomach. Years ago, doctors sometimes removed the entire stomach in Zollinger-Ellison syndrome. This approach is rare today because medications are usually successful in controlling acid production and ulcers.

 
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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