The Zollinger-Ellison syndrome (ZES) is a collection of symptoms and signs (especially, severe peptic ulcer disease) that are caused by tumors called "gastrinomas."
In Zollinger-Ellison syndrome, tumors called gastrinomas produce large amounts of a hormone called gastrin. This is a hormone that increases the amount of stomach acid.
When a large amount of this substance is produced, too much stomach acid is made. This causes ulcers or sores, called "peptic ulcers," to form in the stomach and small intestine.
The primary tumors are usually located in the small intestine usually the first part called the duodenum (approximately 70% are here) with most of the remainder in the pancreas.
The tumor may also be found in nearby lymph nodes and the liver because it is often malignant.
Very rarely (5% or less), the tumor may begin in more distant parts of the body such as the ovaries. The tumors may spread to other parts of the body such as the liver, spleen, lymph nodes, bone, skin and lining of the abdominal cavity.
Most symptoms of Zollinger-Ellison syndrome are caused by the high level of stomach acid and the ulcers that develop as a result.
Symptoms include: abdominal distress, usually located in the area between the breastbone and the navel. The distress can be sharp, burning, or gnawing pain. It can also take the form of a sensation of pressure, fullness, or hunger. The pain typically develops 30 to 90 minutes after a meal. It may be relieved by food, anti-acid medications such as "H-2 blockers (like cimetidine, i.e., Tagamet) or "PPIs" (like omeprazole, i.e., Prilosec), or antacids.heartburn, or gastroesophageal reflux, caused by stomach contents splashing back up into the esophagus. Excess acid may scar the esophagus. This backflow causes pain or burning in the abdomen that travels up toward the throat.diarrhea, which may include stools that are foul smelling from excess fat excretion.weight loss bleeding in the stools. This may turn the stools dark red, black, or make them sticky or "tarry."nausea and vomiting. The vomit may contain blood or may resemble coffee grounds.twenty-five percent of people with ZES also have tumors associated with "MEN-1" or Multiple Endocrine Neoplasia (in the parathyroid or pituitary glands).
Zollinger-Ellison syndrome is caused by a form of cancer known as a gastrinoma.
The excess stomach acid produced by the tumor and the spread of the tumor are responsible for the symptoms and signs of ZES. However, the cause of the gastrinomas is not known.
Some people (about 7%) with ZES have an inherited condition (MEN-1) that results in an increased risk of this tumor.
There are no specific ways to prevent Zollinger-Ellison syndrome.
Suspicion in the following circumstances may lead to an earlier diagnosis ZES: persons with peptic ulcer disease requiring long term treatment or those who have recurring disease after medical treatment. people who have peptic ulcer disease without the bacteria H. pylori. persons who require surgery for complications of peptic ulcer disease. Children or elderly who develop peptic ulcer disease. patients with bad, chronic diarrhea that is unexplained. people who have peptic ulcer disease with either a parathyroid or pituitary tumor.
A person's history (features described above) and physical exam may make a healthcare provider suspect Zollinger-Ellison syndrome.
The most important test to help diagnose this condition is a blood test which measures the level of the hormone gastrin, which is extremely high in someone with ZES (usually 1000 or greater).
A person with ZES also has abnormally increased amounts of stomach acid (a stomach ph of 2.5).
The next is step is to localize the tumor responsible for making excess gastrin and determining whether it has spread. The test of choice in this regard is called a 111In-ocreotide scan. The radioactive part of this scan locks on to receptors in the tumor.
The long-term effects of Zollinger-Ellison syndrome include severe ulcers that may cause a hole in the bowel, blood loss, chronic diarrhea, and weight loss. Death often occurs if the tumor spreads to other parts of the body or cannot be removed.
Zollinger-Ellison is not contagious and does not pose a risk to others.
The ideal treatment of Zollinger-Ellison syndrome is surgery to remove of the tumor or tumors. Once the gastrinoma is located, surgery is advised for a person in whom the tumor has not spread to other parts of the body.
If the cancer has spread to other parts of the body or cannot be located (sometimes the tumors are very small), the treatment is to reduce stomach acid.
Medications such as cimetidine (i.e., Tagamet), omeprazole (i.e., Prilosec) and lansoprazole (i.e., Prevacid) are often used.
In some cases, surgery may be needed to help reduce stomach acid. The surgery removes the stomach which makes the acid.
Chemotherapy may be advised for quickly growing tumors that have spread to other parts of the body. However, this treatment often does not work well.
Surgery may cause bleeding, infection, and allergic reaction to the anesthesia. The most common side effects of the medications used to treat Zollinger-Ellison syndrome include headache, diarrhea, abdominal distress, nausea, dizziness, rash, and cough.
If the gastrinoma is removed with surgery and there is no evidence of spread to other organs, the person may be cured. These people can often lead a normal life free of ulcer symptoms.
However, people in whom the tumor can not be removed surgically may need ongoing medication to reduce stomach acid and to prevent and heal ulcers.
Any new or worsening symptoms should be reported to the healthcare provider. Blood tests and other types of tests may also be used for monitoring in some cases.
Harrison's Principles of Internal Medicine, Fourteenth edition. 1998. McGraw-Hill, pp. 1613-1616
Principles of Ambulatory Medicine, Fourth edition. 1995. Williams&Wilkins, pp. 465-467
Current Medical Diagnosis&Treatment 2000, Lange Medical Books. McGraw -Hill, pp609-610.
