Zollinger Ellison syndrome is a condition in which one or more pancreas tumors form 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 in your stomach,leading to peptic ulcers.
The ulcers that occur as part of Zollinger Ellison syndrome are typically more resistant to treatment than other ulcers, may recur after initial treatment, are often numerous and may occur in unusual areas of your stomach or intestine.
Zollinger-Ellison syndrome is rare. Only a tiny percentage of all people with peptic ulcers have ZE syndrome. It may occur at any age, but it’s more likely to occur in people between the ages of 30 and 60.
The approach to treatment is dual, medications to reduce acid and heal the ulcers, and, if possible, surgery to remove the pancreas tumors.
Zollinger-Ellison Syndrome Symptoms
Zollinger-Ellison syndrome causes signs and symptoms similar to those of a peptic ulcer. These may include:
- Burning, aching, gnawing or discomfort in your upper abdomen
- Black, tarry, foul-smelling stools
- Bloated feeling after meals
- Nausea and vomiting
- Diarrhea
The symptoms may be severe and persistent, and you may find that taking over-the-counter antacids provides you little or no relief, even temporarily.
Causes of Zollinger Ellison Syndrome
Doctors don’t know exactly what causes ZE syndrome. But the sequence of events in ZE syndrome is clear. The syndrome begins when a tumor (gastrinoma) or tumors form in your pancreas or duodenum.
Your pancreas is located behind 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 your level of blood sugar, and 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 are made up of cells that secrete very large amounts of gastrin, which in turn causes excessive acid production in your stomach. As a result, almost everyone with ZE syndrome experiences peptic ulcers.
As well as leading to ulcers, the gastrinomas may sometimes be cancerous (malignant). Gastrinomas grow slowly, but the cancer can spread elsewhere — usually to nearby lymph nodes or your liver.
ZE syndrome may be associated with another disease called multiple endocrine neoplasia type I (MEN I). People with MEN I have multiple tumors in their endocrine system in addition to pancreatic tumors. They also have tumors in their 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.
Treatment for Zollinger-Ellison syndrome
Treatment of Zollinger-Ellison syndrome focuses on both the tumors and the ulcers.
Treatment of pancreas tumors
If you have just one tumor, your doctor may be able to remove it surgically. However, surgery may not be an option if you have numerous tumors or if the tumors have spread to your liver. Often, tumors are small and difficult to locate precisely.
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 an electric current 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
If surgical removal of tumors isn’t an option, medications can help reduce stomach acid. These medications include:
- Proton pump inhibitors.
Most effective medications. Proton pump inhibitors are quite powerful and reduce acid by blocking the action of the tiny “pumps” within acid-secreting cells. Examples of proton pump inhibitors include omeprazole (Prilosec), lansoprazole (Prevacid), rabeprazole (Aciphex), esomeprazole (Nexium) and pantoprazole (Protonix). - Acid blockers.
Also called histamine (H-2) blockers, these medications reduce the amount of hydrochloric acid released into your digestive tract to relieve ulcer pain and encourage healing. Acid blockers 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. Examples of acid blockers your doctor may prescribe may include cimetidine (Tagamet), ranitidine (Zantac), famotidine (Pepcid) and nizatidine (Axid). However, acid blockers don’t work as well as proton pump inhibitors do in treating the ulcers and most people need high, frequent doses of acid blockers for them to be effective.
Your doctor may also suggest one of various surgeries to treat peptic ulcers. This may include surgery to stop an ulcer from bleeding, to relieve an obstruction caused by an ulcer, or to close up the hole 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. This approach is rare today because medications are usually successful in controlling the ulcers.
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