Zollinger Ellison Syndrome

The Zollinger-Ellison syndrome (ZES) is a set of symptoms that are caused by cancerous tumors called gastrinomas.

What is going on in the body?

In Zollinger-Ellison syndrome, tumors called gastrinomas produce large amounts of 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 sores in the lining of the digestive tract, called peptic ulcers, to form in the stomach and small intestine.

The primary tumors are usually located in the pancreas or small intestine. Occasionally they are found in nearby lymph nodes. Very rarely, they can be located 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.

What are the causes and risks of the condition?

Zollinger-Ellison syndrome is caused by a form of cancer known as a gastrinoma. The extra stomach acid produced by the tumor and the spread of the tumor cause the symptoms of ZES. However, the cause of the gastrinomas is not known. Many people with ZES have an inherited condition that results in an increased risk of this tumor.

What are the signs and symptoms of the condition?

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 is often relieved by food or antacids.
• heartburn, or gastroesophageal reflux, caused by stomach contents splashing back up into the esophagus. This backflow causes pain or burning in the abdomen that travels up toward the throat.
• diarrhea, which may include stools that float and are foul smelling
• 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.

A common sign of ZES is abdominal tenderness, especially in the upper abdomen.

How is the condition diagnosed?

A person’s history 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. This blood test measures the level of the hormone gastrin, which is extremely high in someone with ZES. A person with ZES also has abnormally increased amounts of stomach acid. Other special tests may also be needed when ZES is suspected.

Also, special types of x-ray tests and scans can help locate the tumor. Locating the tumor may be difficult.

What can be done to prevent the condition?

There are no specific ways to prevent Zollinger-Ellison syndrome. A person with a strong family history of severe ulcers or certain tumors may have genetic testing for the inherited condition that can increase the risk of ZES. This would not actually prevent the condition, however.

What are the long-term effects of the condition?

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.

What are the risks to others?

Zollinger-Ellison is not contagious and does not pose a risk to others.

What are the treatments for the condition?

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, the treatment is to reduce stomach acid. Medications such as omeprazole and lansoprazole are most commonly used. In some cases, surgery may be needed to help reduce stomach 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.

What are the side effects of the treatments?

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.

What happens after treatment for the condition?

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, many people may need ongoing medication to reduce stomach acid and to prevent and heal ulcers.

How is the condition monitored?

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.