Zollinger Ellison Syndrome Information

Zollinger-Ellison Syndrome, or ZES, is named after two American surgeons, Robert Zollinger and Edwin Ellison. It has been classified as a rare disease by The National Institute of Health's Office of Rare Diseases, afflicting about 1 in one million people in the U.S. each year.

Zollinger-Ellison Syndrome is a condition characterized by the formation of small tumors called gastrinomas. These tumors can form in the pancreas, a gland behind the stomach that is responsible for producing enzymes essential to digesting food, metabolizing protein, carbohydrates and fat. Gastrinomas can also develop in the duodenum, which is found at the top of the small intestine.

Gastrinomas secrete an inordinate amount of gastrin, a hormone controlling the production of hydrochloric acid that helps in digestion. Because gastrin also increases the levels of hydrochloric acid, it becomes the leading cause of peptic ulcers. 95% of patients suffering from the Zollinger-Ellison Syndrome are afflicted with peptic ulcers.

These ulcers usually develop in unusual regions of the esophagus, stomach, intestines, duodenum and jejunum (upper parts of the small intestine), spleen and lymph nodes. They are more painful and difficult to treat compared to other ulcers, and have a tendency to reoccur even after treatment.

The tumors that develop are cancerous in 50% of all ZES cases. They usually occur as part of type 1 multiple endocrine neoplasia, or MEN1, a rare genetic disorder of the endocrine glands which result in numerous tumors all over the body.

The tumors grow slowly, but if they are cancerous, they can spread to other parts of the body like the lymph nodes, the liver, the parathyroid glands, and even the pituitary gland.

Zollinger-Ellison syndrome is usually diagnosed in patients over the age of 50. Doctors have yet to ascertain what exactly causes Zollinger-Ellison Syndrome. It is clear, however, that the condition starts with the formation of a gastrinoma in the pancreas or duodenum.

People suffering from ZES experience all the signs and symptoms of those afflicted with peptic ulcers. These symptoms include heartburn; epigastric (stomach) pain which takes the form of burning, gnawing and aching discomfort in the region of the upper abdomen; nausea and vomiting; diarrhea; loss of weight; jaundice; erosion of the dental enamel, and bleeding from the digestive tract due to duodenum ulceration. Sometimes a patient exhibits occasional hematemesis, or vomiting up of blood.

Other symptoms include the elimination of steatorrhea, non-solid fecal matter which have a tendency to float in excess fat that the intestine and pancreas are unable to absorb. These stools are oily-looking and foul-smelling.

These symptoms can become constant and severe. Taking over-the-counter medications like antacids are unable to provide even temporary relief. Doctors discourage ZES patients from relying on these antacid drugs to ease gastric pain. When used for long periods of time, they may make it harder for the doctor to diagnose Zollinger-Ellison Syndrome.

It is important to consult a doctor immediately at the onset of the first symptoms. Early detection and immediate treatment of Zollinger-Ellison Syndrome is crucial in preventing the cancer from spreading to other parts of the body, like the liver or lymph nodes.

The doctor will start by taking the patient's medical history and examining the patient for any signs and symptoms of the disease. The doctor is likely to order blood tests to be analyzed for any increase in gastrin levels which signals the presence of pancreatic or duodenal tumors.

Another test is an upper gastrointestinal endoscopy, where a tiny lighted video camera (endoscope) is inserted into the throat and into the stomach to search for ulcerations. The endoscope can also be used to take a small sample from the patient's duodenum for laboratory detection of tumors.

The doctor may also examine a patient's stomach and duodenum with an ultrasound probe attached to the end of an endoscope. This technique will allow a closer look at the the digestive tract to be able to locate tumors immediately, and to remove tissue sample for biopsy.

Other less invasive tests include imaging techniques like an ultrasound, a nuclear scan using radioactive tracers, computerized tomography (CT) or magnetic resonance imaging (MRI). These tests will determine the exact location of the gastrinomas.

There are other laboratory tests to make an exact diagnosis for Zollinger-Ellison Syndrome. Some of these include a secretin stimulation test to measure the body's gastrin levels; an antral acidity test; stomach x-rays; a calcium infusion test; and fasting or blood gastrin levels. There are cases where a doctor may recommend an exploratory surgical procedure to determine the location of the tumor for removal.

To treat ZES, doctors will prescribe H2 antagonists like cimetidine (Tagamet) or ranitidine (Zantac) to inhibit the histamine action within the stomach and lower the production of hydrochloric acid. The doctor may also prescribe proton pump inhibitors like lansoprazole (Prevacid) and omeprazole (Prilosec) which also work to slow down acid production. These drugs help to heal the ulcers that have formed in the intestine and duodenum, as well as reduce abdominal pain and incidence of diarrhea.

Another option is to undergo surgery to remove gastrinomas. Doctors will usually recommend surgery, but only if it has been established that the tumors have not spread to other neighboring organs like the lymph nodes or liver.

There are different types of surgery performed on people with Zollinger-Ellison Syndrome. One is surgery to remove the tumors and ulcers, another is stomach removal for more severe cases.

Doctors can perform a debulking, or a removal of gastrinomas in the liver; an embolization, which involves cutting off the tumor's blood supply; radio frequency ablation, which uses heat to eliminate the cancer cells; drugs can also be injected into the tumor to reduce symptoms, or chemotherapy may be used to slow the gastrinoma's growth.

Other surgical procedures are used to prevent an ulcer from bleeding, to remove an ulcerative obstruction, to severe the nerves which stimulate acid production, or to close up the perforations on the stomach walls or duodenum that have been caused by ulcers.

Because the exact cause for ZES has not been established, there is no known method of preventing the disease from occurring.

As long as the gastrinomas have not metastasized and spread to other parts of the body, there is an excellent prognosis for Zollinger-Ellison Syndrome. Morbidity and mortality rates are low, and less than 5% of ZES sufferers develop complications. This is due in part to the much improved medication, surgery and management of the disease.

The cure rate for surgical removal of gastrinomas is only 25% as they reoccur within 5 years. Approximately 50% of ZES sufferers whose tumors were not fully removed during surgery die from symptoms of mestastasis.

Patients suffering the disease as part of the MEN-1 syndrome have very low cure rate, and in cases of malignant gastrinomas that have spread to the liver, a 20 to 30% survival rate of five years has been determined.

However, patients have a longer life expectancy because the tumors grow very slowly. In an advanced metastatic stage, patients who undergo chemotherapy have been found to have a 65% response rate. Surgery and acid-suppressing drugs work effectively in inhibiting acid production and reducing the symptoms of peptic ulcers. Studies have also shown that it will take 5 years for 20 to 40% of ZES sufferers to fully recover from the disease.

In a current study sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases, a study was undertaken to test the effectivity of oral omeprazole in combination with intravenous pantaprazole to treat patients with Zollinger-Ellison Syndrome ulcers.

There have also been current findings in clinical studies which show that the proton pump inhibitor esomeprazole (Nexium) can ensure normal gastric acid levels in ZES sufferers. Twenty-one patients in a controlled trial received several doses of Nexium and were able to maintain normal acid levels with no evidence of gastric of duodenal ulcers in a 12-month period.