In this rare disease, tumors in the digestive tract cause excess production of a certain hormone. High levels of this hormone increase stomach acid, leading to peptic ulcers.
Zollinger-Ellison syndrome is a rare condition in which one or more tumors grow in the pancreas or in the upper part of the small intestine. The tumors are called gastrinomas. These gastrinomas produce large amounts of the hormone gastrin. Gastrin causes the stomach to produce too much acid, which leads to peptic ulcers. High gastrin levels also can cause diarrhea, belly pain and other symptoms.
Zollinger-Ellison syndrome may occur at any time in life. However, people usually find out they have it sometime between ages 20 and 60. Medicines to cut down stomach acid and heal the ulcers are the usual treatment. Some people also may need surgery to remove tumors.
The pancreas is a large organ that lies across your upper abdomen behind your stomach.
Symptoms of Zollinger-Ellison syndrome may include:
See your health care provider if you have a burning, aching pain in your upper belly that won't go away — especially if you also have nausea, vomiting and diarrhea.
Tell your provider if you've been using nonprescription medicines to reduce stomach acid. These include omeprazole (Prilosec, Zegerid), cimetidine (Tagamet HB) or famotidine (Pepcid AC). These medicines may mask your symptoms, which could delay your diagnosis.
The exact cause of Zollinger-Ellison syndrome isn't known. But the pattern of events that occurs in Zollinger-Ellison syndrome typically follows the same sequence. The syndrome begins when one or more tumors form in your pancreas or a part of your small intestine called the duodenum. The duodenum is the section connected to your stomach. Sometimes the tumors form at other sites, such as the lymph nodes next to your pancreas.
Your pancreas sits behind and below your stomach. It makes enzymes that are needed for digesting food. The pancreas also makes many hormones, including insulin. Insulin is the hormone that helps control your blood sugar, also called glucose.
Digestive juices from the pancreas, liver and gallbladder mix in the duodenum. This is where most of your digestion happens.
The tumors that occur with Zollinger-Ellison syndrome are made up of cells that secrete large amounts of the hormone gastrin. For this reason, they are sometimes referred to as gastrinomas. Increased gastrin causes the stomach to make far too much acid. The excess acid then leads to peptic ulcers and sometimes to diarrhea.
Besides causing excess acid production, the tumors are often cancerous. Although the tumors tend to grow slowly, the cancer can spread elsewhere — most commonly to nearby lymph nodes or your liver.
Zollinger-Ellison syndrome may be caused by an inherited condition called multiple endocrine neoplasia, type 1 (MEN 1). People with MEN 1 also have tumors in the parathyroid glands. They may have tumors in their pituitary glands as well.
About 25% of people who have gastrinomas have them as part of MEN 1. They also may have tumors in the pancreas and other organs.
If you have a first-degree relative, such as a sibling or parent, with MEN 1, it's more likely that you'll have Zollinger-Ellison syndrome.
Your health care provider usually bases a diagnosis on the following:
Blood tests. A sample of your blood is examined to see whether you have high gastrin levels. While high gastrin levels may suggest tumors in your pancreas or duodenum, high gastrin levels also can be caused by other conditions. For example, gastrin may be higher if your stomach isn't making acid or if you have had gastric surgery. Taking acid-reducing medicines also can raise gastrin levels.
You need to fast before this test. You also may need to stop taking acid-reducing medicine. Because gastrin levels can vary, this test may be repeated a few times.
You also may have a secretin stimulation test. Secretin is a hormone that regulates gastric acid. For this test, your provider first measures your gastrin levels. You'll then be given an injection of secretin. Your gastrin levels will be measured again. If you have Zollinger-Ellison, your gastrin levels will rise dramatically.
Upper gastrointestinal endoscopy. This test requires sedation. An endoscopy involves putting a thin, flexible instrument down the throat and into the stomach and duodenum. This instrument is called an endoscope. It has a light and a camera at the end of it. It allows your provider to look for ulcers.
During the endoscopy, tissue samples may be removed. This is called a biopsy. The tissue will be examined for gastrin-producing tumors.
Endoscopy also can determine whether the stomach is making acid. If the stomach is making acid and the gastrin level is high, then the diagnosis of Zollinger-Ellison can be established. You will be asked to fast after midnight the night before the test.
Endoscopic ultrasound. This procedure uses an endoscope fitted with an ultrasound probe. The probe makes it easier to spot tumors in your stomach, duodenum and pancreas.
Your provider may remove a tissue sample through the endoscope. This test also requires fasting after midnight and sedation.
Treatment of Zollinger-Ellison syndrome focuses on treating the hormone-secreting tumors as well as the ulcers they cause.
An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have just one tumor, your provider may be able to remove it surgically. But surgery may not be an option if you have many tumors or tumors that have spread to your liver. On the other hand, even if you have multiple tumors, your surgeon still may recommend removing a single large tumor.
In some cases, providers recommend other treatments to control tumor growth, including:
Excess acid production can almost always be controlled. Medicines known as proton pump inhibitors are the first line of treatment. These are effective medicines for controlling acid production in Zollinger-Ellison syndrome.
Proton pump inhibitors are powerful acid-reducing medicines. They work by blocking the action of the tiny "pumps" within acid-secreting cells. Commonly prescribed medicines include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex) and esomeprazole (Nexium).
Long-term use of prescription proton pump inhibitors has been associated with an increased risk of fractures of the hip, wrist and spine. People age 50 and older are at higher risk. However, this risk is small and should be weighed against their acid-blocking benefits.
Octreotide (Sandostatin), a medicine similar to the hormone somatostatin, may counteract the effects of gastrin and be helpful for some people.
Your symptoms may prompt you to visit your primary health care provider first. Your provider will likely refer you to a doctor who specializes in diseases of the digestive system, called a gastroenterologist. You also may be referred to an oncologist. An oncologist is a doctor who specializes in treating cancer.
Here's some information to help you get ready for your appointment and know what to expect.
For Zollinger-Ellison syndrome, some basic questions to ask include:
Your provider is likely to ask you a number of questions, including: