Health Information : Zollinger-Ellison syndrome

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What is Zollinger-Ellison syndrom?
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.

What causes Zollinger-Ellison syndrome?
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.

Treatment of Zollinger-Ellison syndrome
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
An operation to remove the tumors that occur in Zollinger-Ellison requires a skilled surgeon 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)
• A liver transplant
• Attempting to destroy the tumor by cutting off the blood supply (embolization) or by using heat to destroy cancer cells (radiofrequency ablation)
• Injecting drugs into the tumor to relieve cancer symptoms
• Using chemotherapy to try to slow tumor growth

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
Excess acid production can almost always be controlled. Medications known as proton pump inhibitors are the first line of treatment. 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. Commonly prescribed medications include lansoprazole (Prevacid), omeprazole (Prilosec), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium). According to the Food and Drug Administration, long-term use of proton pump inhibitors, especially in people age 50 and older, has been associated with an increased risk of fractures of the hip, wrist and spine.

Your doctor may also suggest one of several operations to treat peptic ulcers, such as 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