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VIPoma

VIPoma is a very rare type of cancer that usually grows from cells in the pancreas called islet cells.

Alternative Names

Vasoactive intestinal peptide-producing tumor; Pancreatic endocrine tumor

Causes, incidence, and risk factors

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

The cause is not known.

VIPomas are often diagnosed in adults, most commonly at age 50. Women are more likely to be affected than men. This cancer is rare. Only about 1 in 10 million people a year get it.

Symptoms

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

Signs and tests

Signs may include:

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

Tests include:

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. Medicines can help control diarrhea. One such medicine is octreotide. It is a manmade form of a natural hormone blocks the action of VIP.

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

Expectations (prognosis)

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

Complications

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

Calling your health care provider

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

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: Elsevier Saunders; 2010:chap 32.

National Cancer Institute. PDQ Pancreatic neuroendocrine tumors (islet cell tumors) treatment. Bethesda, MD: National Cancer Institute. Date last modified 11/10/2012. Available at: http://www.cancer.gov/cancertopics/pdq/treatment/gastric/HealthProfessional. Accessed November 16, 2012.

Updated: 11/17/2012

Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, Stephanie Slon, and Nissi Wang.


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