VIPoma is a rare cancer affecting hormone-producing cells in the pancreas, causing high levels of vasoactive intestinal peptide (VIP). Symptoms include watery diarrhea and dehydration. Treatment is palliative, with surgery providing a cure if the cancer hasn’t spread.
VIPoma is an extremely rare form of cancer, affecting only one in ten million people each year. It affects hormone-producing cells in the pancreas known as islet cells, causing them to produce dangerously high levels of a hormone called vasoactive intestinal peptide (VIP). The cause is not known. Symptoms may include persistent watery diarrhea and dehydration. If the cancer hasn’t spread, the VIPoma can often be cured by removing it; treatment is otherwise palliative, focusing on keeping the patient as comfortable as possible.
VIPoma is also known as Verner Morrison syndrome after the two physicians who first described the disease in 1958; other names include pancreatic endocrine tumor and vasoactive intestinal peptide-producing tumor. It affects women more often than men and is more frequent in patients in their 50s. It is very rare, affecting a small percentage of the population. VIPoma occurs about 90% of the time in the pancreas, but it can also affect other organs including the adrenal glands, colon and liver. The cause is unknown, but some scientists suspect genetics are a factor.
In patients with VIPoma, too much VIP hormone is produced. This hormone relaxes some of the muscles in the gastrointestinal (GI) tract, increases intestinal secretions, and regulates the amount of water in the intestines. The actions of high VIP levels lead to the primary symptom of extreme, persistent watery diarrhea lasting for more than three days. It typically continues in high volumes, even if the patient stops eating, and usually leads to dehydration and hypokalemia or hypokalemia.
Other symptoms of VIPoma include abdominal pain, cramping, nausea, weight loss, and facial flushing. Low blood potassium levels can also cause muscle aches and intense fatigue. Diarrhea and other symptoms can occur periodically for more than a year in many patients before a diagnosis is reached. For this reason, 30-50% of patients’ cancers may have spread and not be curable at the time of diagnosis.
Once a diagnosis of VIPoma is made, the first priority is to correct the dehydration using intravenous (IV) fluids. A drug called octreotide can stop diarrhea and correct other imbalances by blocking the action of VIP. If the cancer is not advanced, complete surgical removal usually provides a cure. For patients whose cancer is incurable, treatment focuses on relieving symptoms and slowing its progression. Life expectancy can range from days to a few months depending on how far the cancer has metastasized.
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