Zollinger-Ellison syndrome

Definition

Zollinger-Ellison syndrome is a condition in which there is increased production of the hormone gastrin. Usually, a small tumor (gastrinoma) in the pancreas or small intestine produces the high levels of gastrin in the blood.

Alternative Names

Z-E syndrome; Gastrinoma

Causes, incidence, and risk factors

Zollinger-Ellison syndrome is caused by tumors, usually found in the head of the pancreas and the upper small intestine. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause production of too much stomach acid.

Gastrinomas occur as single tumors or as small, multiple tumors. About one-half to two-thirds of single gastrinomas are cancerous (malignant) tumors that often spread to the liver and nearby lymph nodes.

Many patients with gastrinomas have several tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients often have tumors of the pituitary gland (brain) and parathyroid gland (neck), as well as tumors of the pancreas.

Symptoms

• Abdominal pain
• Diarrhea
• Vomiting blood (occasional)

Signs and tests

Signs include ulcers in the stomach and small intestine.

Tests include:

• Abdominal CT scan
• Calcium infusion test
• Endoscopic ultrasound
• Exploratory surgery
• Gastrin blood level
• Octreotide scan
• Secretin stimulation test

Treatment

Medications called proton pump inhibitors (omeprazole, lansoprazole, and others) are now the first choice for treating Zollinger-Ellison syndrome. These drugs reduce acid production by the stomach, and promote healing of ulcers in the stomach and small intestine. They also relieve abdominal pain and diarrhea.

Surgery to remove a single gastrinoma may be done if there is no evidence that it has spread to other organs (such as lymph nodes or the liver). Surgery on the stomach (gastrectomy) to control acid production is rarely needed today.

Support Groups

Expectations (prognosis)

Even with early diagnosis and surgery to remove the tumor, the cure rate is relatively low. However, gastrinomas grow slowly, and patients may live for many years after the tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of too much acid production.

Complications

• Failure to locate the tumor during surgery
• Intestinal bleeding or hole (perforation) from ulcers in the stomach or duodenum
• Severe diarrhea and weight loss
• Spread of the tumor to other organs (most often liver and lymph nodes)

Calling your health care provider

Call your health care provider if you have severe abdominal pain that does not go away, especially if it occurs with diarrhea.

Prevention

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References

Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier;2010:chap 32.