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VIPoma

Definition

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

Alternative Names

Vasoactive intestinal peptide-producing tumor; VIPoma syndrome; Pancreatic endocrine tumor

Causes

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 gastrointestinal system.

The exact cause of VIPomas 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. Each year, only about 1 in 10 million people are diagnosed with a VIPoma.

Symptoms

Symptoms of VIPoma may include any of the following:

  • Abdominal pain and cramping
  • Diarrhea (watery, and often in large amounts)
  • Dehydration
  • Flushing or redness of the face
  • Muscle cramps due to low blood calcium (hypokalemia)
  • Nausea
  • Weight loss

Exams and Tests

The health care provider will perform a physical exam and ask about your medical history and symptoms.

Tests that may be done include:

Treatment

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

Support Groups

You can ease the stress of illness by joining a cancer support group. Sharing with others who have common experiences and problems can help you not feel alone.

Outlook (Prognosis)

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

Possible Complications

Complications may include:

  • 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 to 3 days, call your provider.

References

National Cancer Institute. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. Cancer.gov Web site. Updated April 30, 2015. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq#section/all. Accessed December 21, 2016.

Schneider DF, Mazeh H, Lubner SJ, Jaume JC, Chen H. Cancer of the endocrine system. In: Niederhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff's Clinical Oncology. 5th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 71.

Vella A. Gastrointestinal hormones and gut endocrine tumors. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 13th ed. Philadelphia, PA: Elsevier; 2016:chap 38.

Review Date:11/10/2016
Reviewed By:Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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