Rare Hematology News

Disease Profile

VIPoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

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US Estimated

Europe Estimated

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Vasoactive intestinal peptide (VIP) tumor; Pancreatic VIPoma; Vasoactive intestinal peptide-producing tumor;

Categories

Digestive Diseases; Endocrine Diseases; Rare Cancers

Summary

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]

Symptoms

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.

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
Do you have updated information on this disease? We want to hear from you.

Diagnosis

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.

    Treatment

    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.

    Organizations

    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

      Learn more

      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.

          References

          1. Pancreatic neuroendocrine tumors: VIPoma. The American Association of Endocrine Surgeons. https://endocrinediseases.org/neuroendocrine/vipoma.shtml. Accessed 8/2/2018.
          2. Gersten T. VIPoma. MedlinePlus. November 10, 2016; https://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.