Zollinger-Ellison Syndrome

What is Zollinger-Ellison Syndrome (gastrinoma)?

Definition:
Zollinger-Ellison syndrome is a condition caused by abnormal production of the hormone gastrin. A small tumor (gastinoma) 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 bowel. These tumors produce the hormone gastrin and are called gastrinomas. High levels of gastrin cause overproduction of stomach acid.

High stomach 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 occur as single tumors or as small, multiple tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that commonly spread to the liver and nearby lymph nodes. Nearly 25% of patients with gastrinomas have multiple 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 (neck) glands in addition to tumors of the pancreas.

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.
• An octreotide scan shows a tumor in the pancreas or early small bowel.
• Exploratory surgery shows the location of a tumor.
• An endoscopic ultrasound shows a tumor in the pancreas or early small bowel.

Treatment:
A class of acid-suppressing medications called proton pump inhibitors (for example, omeprazole, lansoprazole) is now the first choice of treatment of Zollinger-Ellison syndrome. These drugs dramatically reduce acid production by the stomach, and promote healing of ulcers in the stomach and small bowel. They also provide relief of abdominal pain and diarrhea.

Surgical removal 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). Surgery on the stomach (gastrectomy) to control acid production is rarely necessary today.

Support Groups:

Expectations (prognosis):
Early diagnosis and surgical removal of the tumor is associated with a cure rate of only 20% to 25%. 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 acid overproduction.

Complications:

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

Calling your health care provider:
Call your provider if severe, persistent abdominal pain occurs, especially if it occurs with diarrhea.