Causes

VIPoma causes cells in the pancreas to produce high levels of a hormone called vasoactive intestinal peptide (VIP). This hormone increases secretions from the intestines and relaxes some of the smooth muscles in the GI system.

The cause is not known.

VIPomas are usually diagnosed in adults, most commonly at age 50. Women are more likely to be affected than men. This cancer is rare, affecting an estimated 1 in 10 million people per year.

Symptoms

• Abdominal pain and cramping
• Diarrhea (watery, and often in large amounts)
• Flushing or redness of the face
• Nausea
• Weight loss

Exams and Tests

Signs may include:

• Dehydration
• High volume of diarrhea (even without eating)
• Low stomach acid (achlorhydria)
• Low blood potassium (hypokalemia), which can cause leg cramps

Tests include:

• Blood chemistry tests (basic or comprehensive metabolic panel)
• CT scan of the abdomen
• MRI of the abdomen
• Stool examination for cause of diarrhea and electrolyte levels
• Vasoactive intestinal peptide (VIP) level in the blood

Treatment

The first goal of treatment is to correct dehydration. Fluids are often given through a vein (intravenous fluids) to replace fluids lost in diarrhea.

The next goal is to slow the diarrhea. Some medications can help control diarrhea. Octreotide, which is a human-made form of a natural hormone, blocks the action of VIP.

The best chance for a cure is surgery to remove the tumor. If the tumor has not spread to other organs, surgery can often cure the condition.

Outlook (Prognosis)

Surgery can usually cure VIPomas. However, in one-third to one-half of patients, the tumor has spread by the time of diagnosis and cannot be cured.

Possible Complications

• Cancer spread (metastasis)
• Cardiac arrest from low blood potassium level
• Dehydration

When to Contact a Medical Professional

If you have watery diarrhea for more than 2 - 3 days, call your doctor.

Alternative Names

Vasoactive intestinal peptide-producing tumor; Pancreatic endocrine tumor

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.

National Cancer Institute. Islet cell tumors (pancreatic) treatment PDQ. Updated October 31, 2008.

Updated: 4/28/2012

Reviewed by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.