National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

VIPoma


Other Names:
Vasoactive intestinal peptide (VIP) tumor; Pancreatic VIPoma; Vasoactive intestinal peptide-producing tumor; Vasoactive intestinal peptide (VIP) tumor; Pancreatic VIPoma; Vasoactive intestinal peptide-producing tumor; Diarrheogenic Islet Cell Tumor; VIP-secreting tumor See More
Categories:
Subtypes:
A VIPoma is a rare cancer caused by a type of pancreatic neuroendocrine tumor, which is tumor that arises from cells that produce hormones - allowing the tumor itself to produce hormones. A VIPoma secretes vasoactive intestinal peptide (VIP), a hormone that stimulates the secretion (and inhibits the absorption) of sodium, chloride, potassium and water within the small intestine.[1] Excessive secretion can increase bowel motility and lead to signs and symptoms such as abdominal pain and cramping, severe watery diarrhea, dehydration, flushing of the face, muscle cramps due to low potassium levels (hypokalemia), and weight loss.[1][2] When a person has a VIPoma and these associated symptoms, it is referred to as VIPoma syndrome, also called WDHA syndrome or Verner-Morrison syndrome.[1] The majority of people with a VIPoma have VIPoma syndrome.[3]

VIPomas are usually diagnosed in middle-aged adults or in young children.[3] Tests used to diagnose a VIPoma may include blood tests (including VIP level), imaging studies such as a CT scan or MRI, and examination of a stool sample.[2] Unfortunately, even though these tumors are slow-growing, the majority of VIPomas are metastatic (have spread to other parts of the body) at the time of diagnosis.[1][3] The underlying cause of VIPomas is not yet known.[2]

Management typically first involves treating dehydration with fluids and correcting the imbalance of hormones and other substances (metabolic abnormalities) in the body. Medications such as octreotide and lanreotide can usually stop the diarrhea and inhibit the secretion of VIP.[1][3] Surgery typically follows in order to remove the tumor. When there is no evidence of metastasis, complete removal of the tumor is the only possible cure.[1] The majority of metastatic cases will not be cured by surgery, but symptoms may be managed and prolonged survival is often possible due to the slow-growing nature of these tumors. Targeted therapy or chemotherapy may also be used for people with progressive, advanced disease. The median survival of people with VIPomas is 96 months, but long-term survival mainly depends on the tumor grade, staging, and whether all or most of the tumor can be removed.[3]
Last updated: 8/2/2018

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Hypokalemia
Low blood potassium levels
0002900
Neoplasm of the pancreas
Cancer of the pancreas
Pancreatic tumor
[ more ] 		0002894
Secretory diarrhea 0005208
30%-79% of people have these symptoms
Anorexia 0002039
Chronic fatigue
Chronic extreme exhaustion
0012432
Dehydration 0001944
Diabetes mellitus 0000819
Episodic abdominal pain 0002574
Erythema 0010783
Generalized muscle weakness 0003324
Hepatomegaly
Enlarged liver
0002240
Hypercalcemia
High blood calcium levels
Increased calcium in blood
[ more ] 		0003072
Malabsorption
Intestinal malabsorption
0002024
Muscle spasm 0003394
Nausea and vomiting 0002017
Normochromic anemia 0001895
Poor appetite
Decreased appetite
0004396
Weight loss 0001824
5%-29% of people have these symptoms
Abnormal gastrointestinal motility 0030895
Ascites
Accumulation of fluid in the abdomen
0001541
Extrahepatic cholestasis 0012334
Intermittent jaundice
Intermittent yellow skin
Intermittent yellowing of skin
[ more ] 		0001046
Intrahepatic cholestasis 0001406
1%-4% of people have these symptoms
Adrenocortical adenoma 0008256
Benign gastrointestinal tract tumors
Non-cancerous GI tumors
0006719
Elevated calcitonin 0003528
Follicular thyroid carcinoma 0006731
Ganglioneuroma 0003005
Growth hormone excess 0000845
Increased circulating cortisol level 0003118
Increased circulating gonadotropin level
Elevated gonadotropins
Elevated serum gonadotropins
Gonadotropin excess
[ more ] 		0000837
Increased circulating prolactin concentration 0000870
Neoplasm of the liver
Liver cancer
Liver tumor
[ more ] 		0002896
Parathyroid adenoma 0002897
Pituitary adenoma
Noncancerous tumor in pituitary gland
0002893
Primary hyperparathyroidism 0008200
Respiratory insufficiency due to muscle weakness
Decreased lung function due to weak breathing muscles
0002747
Subcutaneous lipoma 0001031
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Last updated: 7/1/2020

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

  • Orphanet lists international laboratories offering diagnostic testing for this condition.

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnoses include all other causes of chronic diarrhea such as malabsorption syndrome, Crohn disease, ulcerative colitis, microscopic colitis (see these terms), and gastrointestinal infections.
Visit the Orphanet disease page for more information.

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to VIPoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Merck Manual for health care professionals provides information on VIPoma.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss VIPoma. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Pancreatic neuroendocrine tumors: VIPoma. The American Association of Endocrine Surgeons. http://endocrinediseases.org/neuroendocrine/vipoma.shtml. Accessed 8/2/2018.
  2. Gersten T. VIPoma. MedlinePlus. November 10, 2016; http://www.nlm.nih.gov/medlineplus/ency/article/000228.htm.
  3. Bergsland E. VIPoma: Clinical manifestations, diagnosis, and management. UpToDate. Waltham, MA: UpToDate; January 31, 2018; https://www.uptodate.com/contents/vipoma-clinical-manifestations-diagnosis-and-management.