VIPoma

Synonyms

3

Overview

A rare disorder caused by an increase in secretion of vasoactive intestinal peptide (VIP) by the pancreas. The syndrome is often caused by an islet-cell tumor (except for beta cells) in the pancreas.

Symptoms

  • Diarrhoea
  • Watery stools
  • Abdominal pain
  • Back pain
  • Flushing
  • Sweating
  • Failure to thrive
  • Colonic dilatation
  • Diarrhoea
  • Watery stools
  • Abdominal pain
  • Back pain
  • Flushing
  • Sweating
  • Failure to thrive
  • Colonic dilatation
  • Watery diarrhea
  • Hypokalemia
  • Absence of hydrochloric acid in digestive juices
  • Potassium loss
  • Metabolic acidosis

Causes

The cause of VIPoma is unknown.

Evidence suggests that pancreatic cancer is linked to inhalation or absorption of the following carcinogens , which are then excreted by the pancreas:

  • Cigarettes
  • Food additives
  • Industrial chemicals, such as beta-naphthalene, benzidine, and urea.

Possible predisposing factors are chronic pancreatitis, diabetes mellitus, and chronic alcohol abuse (both pancreatitis and diabetes mellitus may be early manifestations of the disease as well). Pancreatic cancer incidence increases with age, peaking between ages 60 and 70. Geographically, the incidence is highest in Israel, the United States, Sweden, and Canada.

Prevention

Because there is no known cause, the best line of prevention is regular screening and maintaining good overall health. Patients who have one or more risk factors, or who have previously been treated for endocrine tumors, should be tested at least once or twice a year.

Diagnosis

Signs of the disease are:

  • High level of VIP (vasoactive intestinal peptide) in the blood
  • High volume of diarrhea (continues despite fasting)

Fasting is one way to test for VIPoma.

The physician may also request that the stool be collected and analyzed to determine electrolyte content.

A CT scan or MRI may be ordered to determine the location of the tumor.

source: HowStuffWorks

Prognosis

If the cancer is localized, VIPoma can be cured by surgically removing the tumor. However, about half of VIPoma cases have spread by the time they are diagnosed and are no longer curable. In such cases, the life expectancy is anywhere from a few days to a few months, depending on the degree of metastasis.

source: KnowCancer

Treatment

The only curative option for VIPoma is surgery to remove the tumor, or in some cases, the entire pancreas (pancreatectomy). This presents a complete cure only if the tumor is confined to the pancreas. If the case is incurable, treatments are geared towatds relieving the symptoms and prevent further spread.

When palliative treatment is involved, the first goal is to prevent dehydration. Intravenous (IV) fluids are usually administered to replace the liquids lost in diarrhea. The next step is slowing the diarrhea itself. Octreotide, a synthetic version of the natural hormone somatostatin, blocks the action of VIP and reduces water loss.

Resources

  • NIH
  • HowStuffWorks
  • KnowCancer