Zollinger-Ellison syndrome is a rare disease that usually occurs when a tumor called gastrinoma develops in the pancreas or duodenum. A gastrinoma secretes a hormone known as gastrin, which results in an overproduction of acid in the stomach. Gastrinomas resulting in Zollinger-Ellison syndrome may be caused by the pancreas or less likely the small intestine. Sometimes they may originate from other organs in the body, such as lymph nodes, stomach, liver and ovaries.
According to the National Institute of Diabetes and Digestive and Renal Diseases (NIDDK), Zollinger-Ellison syndrome is rare. Approximately one in every million people will develop the syndrome, but it is most common among men aged 30 to 50 years. Someone with Zolllinger-Ellison syndrome will probably develop severe, recurrent ulcers of the duodenal and jejunum ulcers, which are the recurrent esophagus, stomach and upper parts of the small intestine. Ulcers are caused by excessive acidity.
A person with Zollinger-Ellison syndrome develops tumors in the endocrine and pancreas that cause symptoms similar to those of a peptic ulcer. Most people with Zollinger-Ellison syndrome have multiple tumors in the endocrine system and tumors in the pancreas. Signs and symptoms are similar to those of a peptic ulcer and are as follows:
• Upper abdominal discomfort
• Burning and pain in the upper abdomen
• Bleeding in the digestive tract
• General weakness
• Black, “tar” stool, bleeding in the digestive tract
• Unintentional weight loss
• Anorexia or feeling too full quickly
Some people may experience heartburn or gastroesophageal reflux when they return to the esophagus if stomach acid and food from the stomach are heavy.
It is not entirely clear what causes Zollinger-Ellison syndrome or developing gastrinomas. Gastrinomas secrete an excessive amount of gastrin in the stomach and duodenum, resulting in too much stomach acid. Over time, this additional acid causes peptic ulcers to form in the lining of the duodenum. The result can be multiple ulcers, which can cause pain or upper gastrointestinal bleeding.
In addition to being the cause of excessive acid production, cancerous or gastrinomas may also be malignant. Cancer can most commonly spread to nearby lymph nodes or other parts of the liver. Most people develop Zollinger-Ellison syndrome for no apparent reason. However, a genetic condition called multiple endocrine neoplasia type 1 (MEN1) is responsible for 25 to 30 percent of cases. MEN1 also causes multiple endocrine cancers. A person has a 50 percent chance of developing Zollinger-Ellison syndrome from a parent with the syndrome. An individual may be at risk of developing a gastrinoma if they have several family members with endocrine cancer or if they have a family member with MEN1.
Doctors can do some tests by asking the person for signs and medical history. Tests are as follows:
Blood tests: High levels of gastrin in the blood may indicate a tumor in the pancreas or intestine of a person. Before starting the blood test, one should avoid:
• Avoid using acid-lowering medications for a period of time prescribed by the doctor • They
may need to repeat the blood test at least three times as gastrin levels may fluctuate.
Stomach acidity level: A doctor will ask for tests to find out why you have a high level of gastrin, and then start treatment. People with chronic gastric inflammation and recent gastric surgery may have a high level of gastrin in the blood, even if the stomach does not produce much acid. It is important for the doctor to identify why the person has high gastrin levels in order to find the right treatment. They can test the acidity of the stomach. If the stomach acidity is not high, the person is unlikely to have Zollinger-Ellison syndrome. If the stomach produces too much acid, a doctor may recommend a secretin stimulation test. This includes measuring gastrin levels, injecting the secretory hormone, and re-measuring gastrin levels. If the gastrin levels rise, this indicates the presence of Zollinger-Ellison.
Upper gastrointestinal endoscopy: An upper gastrointestinal endoscopy involves a doctor placing an endoscope to look for an ulcer into the mouth of a person, down the esophagus, into the stomach and duodenum. An endoscope is a long and thin tube with a light and video camera that allows the doctor to see what is going on in certain parts of the body. Other names given for the procedure are as follows:
• Upper endoscopy
• Upper GI endoscopy
• Esophagogastroduodenoscopy (EGD) The
doctor may also take a biopsy or tissue sample to test for gastrin-producing tumors from the duodenum. Before this procedure, the doctor will recommend fasting for a certain period of time.
Imaging scans: A health professional can locate tumors using the following methods:
• A nuclear scan
• An MRI scan or CT scan
• An ultrasound scan In
endoscopic ultrasound, the doctor places an endoscope with an ultrasound device inside the body. This allows them to see inside the stomach and intestines. Internal ultrasound helps to detect tumors and to take tissue samples. Individuals must fast for a while before this procedure. Angiography may help to locate tumors in the pancreas. The doctor places a catheter with a flexible tube in the arteries near the pancreas. A healthcare professional then injects contrast dye into the blood vessel through the catheter. The dye appears on X-ray images that emphasize blood vessels. They tend to be more intense in tumors.
Treatment of Zollinger-Ellison syndrome focuses on tumors and ulcers. Various treatment types are available. Doctors are usually treat tumors before and other forms of therapy as follows:
prevention of tumor growth
may include treatment options are to stop the tumor growth:
• Chemotherapy of tumors to slow the growth rate of
• the receipt of Gastrinoma the liver can prevent the development of liver and other tumors
• Embolization or cut off the blood supply to tumors
• Drugs to injected directly into the tumor
to reduce the production of gastrin
doctor may also use a hormone drug called octreotide to control the symptoms. Octreotide is a drug that can reduce gastrin production.
Reducing acid levels
Taking medication to control excess acid can increase ulcers’ chances of recovery, resulting in fewer symptoms. The first line of treatment for the treatment of excess acid is a proton pump inhibitor (PPI). This is a type of drug that helps control excess acid production. PPIs reduce acid by blocking the effect of small pumps in acid secreting cells. Examples of PPI include:
• Esomeprazole (Nexium)
• Omeprazole (Prilosec)
• Lansoprazole (Prevacid)
• Pantoprazole (Protonix)
• Rabeprazole (Aciphex)
People with chronic Zollinger-Ellison syndrome may use pantoprazole delayed-release tablets as a long-term treatment option. If stomach acid levels fall, peptic ulcers have a better chance of healing and the symptoms of Zollinger-Ellison syndrome will be less.
Tumors can be difficult to remove because they tend to be small and difficult to locate. A surgeon can remove a single tumor, but may not be able to operate if several tumors are present or spread to the liver. According to the National Organization for Rare Disorders (NORD), successful removal of a gastrinoma occurs in about 20-30 percent of cases. If a person has a peptic ulcer and the doctor may recommend surgery in the following cases:
• Closure of any damage or perforation of the stomach or duodenum wall
• To relieve the ulcer-induced blockage
• To stop bleeding
• In rare and severe cases, a surgeon can remove the stomach.
Most cases of Zollinger-Ellison syndrome are sporadic. This means that it is not caused by any other situation and it is not possible to predict who will receive it or ways of preventing it. However, if the family has a history of MEN1 gene mutation, the doctor may recommend that a person undergo predictive testing, even if he or she does not have any symptoms. At the age of 20, a person with a MEN1 gene mutation has a 50% chance of catching symptoms. According to the Genetic and Rare Diseases Information Center (GARD), the chance of developing symptoms increases to 95 percent at the age of 40. If a person does not show symptoms until the age of 40, it is likely that he or she will not have the MEN1 mutation. So far, there is no evidence that the diet affects the risk of developing the syndrome.
How Does the Digestive System Work?
Stomach, duodenum and pancreas are part of the digestive system. Stomach and pancreas secrete stomach, which stimulates the production of gastric acid and other digestive juices that help break down food. Gastrin circulates in the bloodstream and sends signals to other stomach cells to release gastric acid. Food is transported to the duodenum, the first part of the small intestine. There, secretions are further deteriorated. The small intestine is a tubular organ located between the large intestine and the stomach. In a healthy system, stomach cells control how much gastrin a person produces. This prevents excessive gastric acid formation. People with Zollinger-Ellison syndrome may have an impairment in their balance because gastrinomas produce extra gastrin.