Alternate Names: Z-E syndrome; Gastrinoma

Causes and Risks:

Zollinger-Ellison syndrome is caused by tumors usually found in the head of the pancreas and the upper small bowel. These tumors over produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid.

High acid levels lead to multiple ulcers in the stomach and small bowel. Patients with Zollinger-Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel.

Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25% of patients with gastrinomas have multiple tumors as part of a condition called Type I multiple endocrine neoplasia (MEN I).MEN I patients have tumors in their pituitary gland and parathyroid glands in addition to tumors of the pancreas.

Prevention:

There is no known way to prevent this tumor.

Symptoms:

• pain
• vomiting blood (occasional)
• diarrhea

Signs and Tests:

• The gastrin level is increased.
• A secretin stimulation test is positive.
• A calcium infusion test is positive.
• An abdominal CT scan shows a tumor (or tumors) in the pancreas or early small bowel.

Treatment:

A class of acid-suppressing medications called proton pump inhibitors (for example, omeprazole, lansoprazole) is now first line treatment of Zollinger-Ellison syndrome.These drugs reduce acid production by the stomach dramatically and promote healing of ulcers in the stomach and small bowel.They also provide relief of abdominal pain and diarrhea.Another class of acid-suppressing medications called H-2 blockers may also be used (for example, cimetidine, ranitidine).The H-2 blockers are less potent than the proton-pump inhibitors.

Surgical resection of a single gastrinoma may be attempted if there is no evidence that it has spread to other organs (such as lymph nodes or the liver).Resection of the stomach (gastrectomy) to control acid over production is rarely if ever necessary today.

Prognosis:

Early diagnosis and surgical resection is associated with a cure rate of only 20-25 %.Gastrinomas are slow growing, and patients may live for many years after the tumor is discovered. Acid-suppressing medications are very effective at controlling the symptoms of acid over production.

Complications:

• spread of the tumor to other organs (most often liver and lymph nodes)
• failure to locate the tumor during surgery

Call your health care provider if or if severe, persistentabdominal pain occurs, especially if it occurs with diarrhea.