VIPoma

Title

Other Names:

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

Categories:

Digestive Diseases; Endocrine Diseases; Rare Cancers

Summary Summary

VIPoma is a rare cancer that develops within the pancreas. This tumor causes pancreatic cells to produce high levels of a hormone called vasoactive intestinal peptide (VIP). The signs and symptoms of a VIPoma include abdominal pain, flushing or redness of the face, nausea, watery diarrhea, weight loss, dehydration, and low blood potassium (hypokalemia). VIPomas are usually diagnosed in adults around age 50. The cause of VIPoma is unknown. Treatment may include intravenous (IV) fluids to correct dehydration, medications such as octreotide to help control diarrhea, and surgery to remove the tumor.^[1]

Last updated: 3/8/2010

Symptoms 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.

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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 cramps		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
Neoplasm of the liver	Liver cancer Liver tumor [ more ]	0002896
Parathyroid adenoma		0002897
Pituitary adenoma		0002893
Primary hyperparathyroidism		0008200
Prolactin excess		0000870
Respiratory insufficiency due to muscle weakness		0002747
Subcutaneous lipoma		0001031

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 5/1/2018

Diagnosis 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 Treatment

Treatment for VIPoma may include intravenous (IV) fluids to correct dehydration, medications such as octreotide to help control diarrhea, and surgery to remove the tumor.^[1] If the tumor has spread (metastasized) to the liver or other tissues, treatment may involve chemotherapy, radiofrequency ablation, or hepatic artery embolization.^[2]^[3]^[4]^[5]

Last updated: 3/8/2010

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.

Octreotide (Brand name: Sandostatin LAR) - Manufactured by Novartis Pharmaceuticals Corporation
FDA-approved indication: Reduction of growth hormone and IGF-1 (somatomedin C) in acromegaly.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

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

Research Research

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.

Learn More 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

MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.

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 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.

GARD Answers GARD Answers

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. Submit a new question

My husband was diagnosed in 1998 with VIPoma. He uses octreotide to help control the symptoms. Is there any other known treatment (other than surgery) for this rare cancer? See answer

Have a question? Contact a GARD Information Specialist.

References References

Stitham SO. VIPoma. MedlinePlus Web site. September 4, 2008; http://www.nlm.nih.gov/medlineplus/ency/article/000228.htm. Accessed 3/8/2010.
Song S, Shi R, Li B, Liu Y. Diagnosis and treatment of pancreatic vasoactive intestinal peptide endocrine tumors. Pancreas. 2009 Oct;38(7):811-4. http://www.ncbi.nlm.nih.gov/pubmed/19657309 .
Ghaferi AA, Chojnacki KA, Long WD, Cameron JL, Yeo CJ. Pancreatic VIPomas: subject review and one institutional experience. J Gastrointest Surg. 2008 Feb;12(2):382-93. http://www.ncbi.nlm.nih.gov/pubmed/17510774 .
Moug SJ, Leen E, Horgan PG, Imrie CW. Radiofrequency ablation has a valuable therapeutic role in metastatic VIPoma. Pancreatology. 2006;6(1-2):155-159 http://www.ncbi.nlm.nih.gov/pubmed/16354964.
Case CC, Wirfel K, Vassilopoulou-Sellin R. Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report. Med Oncol. 2002;19(3):181-187 http://www.ncbi.nlm.nih.gov/pubmed/12482130.