Description

The Zollinger-Ellison syndrome is a condition in which one or more to the growth of tumors in the pancreas or small intestine. The tumors, called gastrinomas, produce large amounts of the hormone gastrin. Gastrin causes the stomach to produce too much acid, which leads to peptic ulcers. High levels of gastrin can also cause diarrhea, abdominal pain and other symptoms.

The Zollinger-Ellison syndrome is rare. Although it can occur at any time of life, people often find that they have at some time between the ages of 20 and 50. Medications to reduce acid in the stomach and cure ulcers are the usual treatment. Some people may also need surgery to remove the tumors.

Symptoms

The symptoms of Zollinger-Ellison syndrome may include:

  • Stomach pain.
  • The diarrhea.
  • Burning, pain or discomfort in the upper abdomen.
  • Acid reflux and heartburn.
  • The belching.
  • Nausea and vomiting.
  • Bleeding in the digestive tract.
  • Losing weight without trying.
  • Loss of appetite.

When to see a doctor

Consult a health care professional if you have a burning sensation, pain in the upper abdomen that does not go away — especially if you also have nausea, vomiting, and diarrhea.

Tell your health care professional if you have been the use of over-the-counter medications to reduce stomach acid. These include omeprazole (Prilosec, Zegerid), cimetidine (Tagamet HB) or famotidine (Pepcid AC). These medications may mask the symptoms, which can delay its diagnosis.

Causes

The exact cause of the Zollinger-Ellison syndrome is not known. But the pattern of events that occurs in Zollinger-Ellison syndrome usually follows the same sequence. The syndrome begins when one or more tumors form in the pancreas or a part of the small intestine called the duodenum. The duodenum is the section connected with the stomach. Sometimes, the tumors form in other sites, such as the lymph nodes near the pancreas.

The pancreas is located behind the stomach. Makes enzymes which are necessary for the digestion of food. The pancreas also produces many hormones, including insulin. Insulin is the hormone that helps control blood sugar, also called glucose.

The digestive juices from the pancreas, liver and gallbladder mix in the duodenum. Here is where most of the digestion occurs.

The tumors that form with the Zollinger-Ellison syndrome are composed of cells that secrete large amounts of the hormone gastrin. For this reason, they are sometimes called gastrinomas. The increase in gastrin causes the stomach to make too much acid. The excess of acid then leads to peptic ulcers and sometimes diarrhea.

In addition cause excess acid production, the cancerous tumors. Although the tumors tend to grow slowly, the cancer can spread to other places, most often to nearby lymph nodes or the liver.

Association with MEN 1

The Zollinger-Ellison syndrome can be caused by a hereditary 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.

Approximately 25% of the people who have gastrinomas have as part of the MEN 1. They may also have tumors in the pancreas and other organs.

Risk factors

If you have a first-degree relative, like a brother or father, with the MEN 1, it is more likely that you are going to have the Zollinger-Ellison syndrome.

Diagnosis

A health professional is usually the basis of a diagnosis in the following:

  • History of medicine. A healthcare provider will usually ask about your symptoms and review your medical history.
  • Blood tests.A sample of blood is examined for high levels of gastrin. While high levels of gastrin may suggest that the tumors in the pancreas or the duodenum, the high levels of gastrin can also be caused by other conditions. For example, gastrin may be higher if your stomach is not doing acid or if you have had gastric surgery. Taking acid-reducing medications can also raise the levels of gastrin. You need to fast before this test. It is also possible that you may need to stop taking acid-reducing medicine. Because gastrin levels may vary, this test may be repeated a couple of times. You can also have a secretin stimulation test. Secretin is a hormone that regulates the production of gastric acid. For this test, a medical professional first measurements of their levels of gastrin. Then it is given an injection of secretin. Your gastrin levels will be measured again. If you have Zollinger-Ellison syndrome, their gastrin levels will increase dramatically. Your blood may also be tested by by a protein called chromogranin a, which is high in most people with gastrinomas.
  • Upper gastrointestinal endoscopy.This test requires sedation. An endoscopy involves putting a thin, flexible instrument down the throat and into the stomach and the duodenum. This instrument called an endoscope. It has a light and a camera on the end of the same. Allows a medical professional to look for ulcers. During the endoscopy, tissue samples can be removed. This is called a biopsy. The tissue is examined for the gastrin-producing tumors. Endoscopy can also find out if the stomach is doing acid. If the stomach is doing acid and gastrin level is high, then the diagnosis of Zollinger-Ellison syndrome can be established. You will be asked to fast after midnight the night before the test.
  • Endoscopic ultrasound.This procedure uses an endoscope equipped with an ultrasound probe. The probe makes it easier to detect tumors in the stomach, the duodenum and the pancreas. Your healthcare provider can remove a sample of tissue through the endoscope. This test also requires fasting after midnight, and sedation.
  • Imaging tests. Imaging techniques can be used to diagnose tumors, such as ct, mri and Ga-DOTATATE PET-CT.

Blood tests. A sample of blood is examined for high levels of gastrin. While high levels of gastrin may suggest that the tumors in the pancreas or the duodenum, the high levels of gastrin can also be caused by other conditions. For example, gastrin may be higher if your stomach is not doing acid or if you have had gastric surgery. Taking acid-reducing medications can also raise the levels of gastrin.

You need to fast before this test. It is also possible that you may need to stop taking acid-reducing medicine. Because gastrin levels may vary, this test may be repeated a couple of times.

You can also have a secretin stimulation test. Secretin is a hormone that regulates the production of gastric acid. For this test, a medical professional first measurements of their levels of gastrin. Then it is given an injection of secretin. Your gastrin levels will be measured again. If you have Zollinger-Ellison syndrome, their gastrin levels will increase dramatically.

Your blood may also be tested by by a protein called chromogranin a, which is high in most people with gastrinomas.

Upper gastrointestinal endoscopy. This test requires sedation. An endoscopy involves putting a thin, flexible instrument down the throat and into the stomach and the duodenum. This instrument called an endoscope. It has a light and a camera on the end of the same. Allows a medical professional to look for ulcers.

During the endoscopy, tissue samples can be removed. This is called a biopsy. The tissue is examined for the gastrin-producing tumors.

Endoscopy can also find out if the stomach is doing acid. If the stomach is doing acid and gastrin level is high, then the diagnosis of Zollinger-Ellison syndrome can be established. You will be asked to fast after midnight the night before the test.

Endoscopic ultrasound. This procedure uses an endoscope equipped with an ultrasound probe. The probe makes it easier to detect tumors in the stomach, the duodenum and the pancreas.

Your healthcare provider can remove a sample of tissue through the endoscope. This test also requires fasting after midnight, and sedation.

Treatment

Treatment of Zollinger-Ellison syndrome focuses on the treatment of hormone-secreting tumors as well as ulcers that cause.

The treatment of tumors

An operation to remove the gastrinomas requires a skilled surgeon because the tumors are often small and difficult to find. If you have a tumor, a medical professional 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 the liver. On the other hand, even if you have multiple tumors, your doctor may still recommend the removal of a single large tumor.

In some cases, physicians may recommend other treatments to control the growth of the tumor, including:

  • Remove a tumor in the liver as possible, a process known as debulking.
  • Trying to destroy the tumor by cutting off the blood supply, called embolization.
  • The use of heat to destroy cancer cells with a procedure called radiofrequency ablation.
  • The injection of drugs into the tumor to relieve the symptoms of cancer.
  • The use of chemotherapy to reduce the tumor growth.
  • To have a liver transplant.

The treatment of excess acid

The excess production of acid can almost always be controlled. Drugs known as inhibitors of the proton pump are the first line of treatment. These are effective medications for the control of the production of acid in the Zollinger-Ellison syndrome.

Of the proton pump are potent inhibitors of acid-reducing medication. They work by blocking the action of the small "pumps" within acid-secreting cells. Commonly prescribed medications include lansoprazole (Prevacid), omeprazole (Prilosec, Zegerid), pantoprazole (Protonix), rabeprazole (Aciphex), and esomeprazole (Nexium). Talk with your healthcare provider about the risks of taking these drugs long-term.

Octreotide (Sandostatin), a drug similar to the hormone somatostatin, may counteract the effects of gastrin and be useful for some people.

Preparing for your appointment

Your symptoms may be asked to visit your primary healthcare professional in the first place. Your health care professional is likely to see a doctor that specializes in diseases of the digestive system, called a gastroenterologist. You may also be referred to an oncologist. An oncologist is a physician who specializes in the treatment of cancer.

Here's some information to help you prepare for your appointment and know what to expect.

What you can do

  • Be aware of any pre-appointment restrictions. When you make your appointment, let your care team know if you are taking any of the medicines. Certain acid-reducing drugs, such as inhibitors of the proton pump or H-2 blockers, may affect the results of some tests are used to diagnose the syndrome of Zollinger-Ellison syndrome. However, do not stop taking these medicines without checking with your care professional first.
  • Write down any symptoms you're experiencing, including any that may seem unrelated.
  • Write down key personal information, including any major stresses or recent life changes. Also write what you know about the history of your family.
  • Make a list of all medications, vitamins or supplements you are taking.
  • Write questions during your appointment.

Questions to ask your doctor

For Zollinger-Ellison syndrome, some basic questions to ask include:

  • What is the most likely cause of my symptoms?
  • Is there some other explanation for the symptoms?
  • What evidence do I need to confirm the diagnosis? How should I prepare for the exams?
  • What treatments are available for the Zollinger-Ellison syndrome, and what do you recommend for me?
  • There are restrictions in the diet should I follow?
  • How often should I return for follow-up appointments?
  • What is my prognosis?
  • I need to see a specialist?
  • Is there a generic alternative to the medicine you're prescribing for me?
  • There are web sites that we recommend to learn more about the Zollinger-Ellison syndrome?
  • Other medical problems more likely to happen because I have the Zollinger-Ellison syndrome?

What to expect from your doctor

You'll likely be asked a few questions during the appointment, including:

  • When did you begin experiencing symptoms?
  • Do you have symptoms all the time, or come and go?
  • How bad are the symptoms?
  • Is there something to improve the symptoms?
  • Have you noticed anything that makes your symptoms worse?
  • Have you ever been told that you have a stomach ulcer? How is it diagnosed?
  • Do you or anyone in your family ever been diagnosed with multiple endocrine neoplasia type 1?
  • Do you or someone in your family has been diagnosed with parathyroid, thyroid or pituitary problems?
  • Have you ever been told that you have high calcium in the blood?
Symptoms and treatment of Zollinger-Ellison syndrome