# VIPoma

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Tumor that often causes a syndrome of diarrhea and electrolyte imbalance

Medical condition

VIPoma Specialty Oncology medicine Symptoms watery diarrhea, hypokalemia, achlorhydria. Usual onset most patients are middle aged Types usually solitary, 2/3 are malignant Prognosis bad. Deaths frequent.

A **VIPoma** or **vipoma** ([/vɪˈpoʊmə/](https://en.wikipedia.org/wiki/Help:IPA/English)) is a rare [endocrine tumor](/source/Endocrine_oncology)[1] that overproduces [vasoactive intestinal peptide](/source/Vasoactive_intestinal_peptide) (thus *VIP* + *[-oma](https://en.wiktionary.org/wiki/-oma#Suffix)*). The [incidence](/source/Incidence_(epidemiology)) is about 1 per 10,000,000 per year. 90% of VIPomas originate from the [non-β islet cells](/source/Pancreatic_islets) of the [pancreas](/source/Pancreas), sometimes associated with [multiple endocrine neoplasia type 1](/source/Multiple_endocrine_neoplasia_type_1). Roughly 50–75% of VIPomas are [malignant](/source/Cancer), but even when they are [benign](/source/Benign_tumor), they are problematic because they tend to cause a specific [syndrome](/source/Syndrome): the massive amounts of secreted VIP overstimulates pancreatic bicarbonate and chloride secretion, and its binding to intestinal epithelial cells leads to sodium, chloride and water secretion into the bowel (secretory watery diarrhea, often >3L/day). all this causes a [syndrome](/source/Syndrome) of profound and chronic watery [diarrhea](/source/Diarrhea) and resultant [dehydration](/source/Dehydration), [hypokalemia](/source/Hypokalemia), [achlorhydria](/source/Achlorhydria), acidosis, [flushing](/source/Flushing_(physiology)) and [hypotension](/source/Hypotension) (from [vasodilation](/source/Vasodilation)), [hypercalcemia](/source/Hypercalcemia), and [hyperglycemia](/source/Hyperglycemia).[2][3] This syndrome is called **Verner–Morrison syndrome** (**VMS**), **WDHA syndrome** (from watery diarrhea–hypokalemia–achlorhydria), or **pancreatic cholera syndrome** (**PCS**). The [eponym](/source/Medical_eponyms) reflects the physicians who first described the syndrome.[4]

## Symptoms and signs

The major clinical features are prolonged watery [diarrhea](/source/Diarrhea) (fasting stool volume > 750 to 1000 mL/day) and symptoms of [hypokalemia](/source/Hypokalemia) and [dehydration](/source/Dehydration). Half of the patients have relatively constant [diarrhea](/source/Diarrhea) while the rest have alternating periods of severe and moderate [diarrhea](/source/Diarrhea). One third have diarrhea < 1yr before diagnosis, but in 25%, diarrhea is present for 5 yr or more before diagnosis. [Lethargy](/source/Lethargy), muscle weakness, [nausea](/source/Nausea), vomiting and crampy abdominal pain are frequent symptoms. [Hypokalemia](/source/Hypokalemia) and impaired [glucose](/source/Glucose) tolerance occur in < 50% of patients. [Achlorhydria](/source/Achlorhydria) is also a feature. During attacks of diarrhea, flushing similar to the [carcinoid syndrome](/source/Carcinoid_syndrome) occur rarely.[5]

## Diagnosis

Besides the clinical picture, [fasting](/source/Fasting) VIP [plasma](/source/Blood_plasma) level may confirm the diagnosis, and [CT scan](/source/CT_scan) and [somatostatin](/source/Somatostatin) receptor [scintigraphy](/source/Scintigraphy) are used to localise the [tumor](/source/Tumor), which is usually [metastatic](/source/Metastasis) at presentation.[6]

Tests include:

- Blood chemistry tests (basic or comprehensive metabolic panel)

- CT scan of the abdomen

- MRI of the abdomen

- Stool examination for the cause of diarrhea and electrolyte levels

- Vasoactive intestinal peptide (VIP) level in the blood[6]

## Treatment

The first goal of treatment is to correct dehydration. Fluids are often given intravenously to replace those lost during diarrhea. The next goal is to slow the diarrhea. Some medications can help control it. [Octreotide](/source/Octreotide), an artificially synthesized form of somatostatin (a naturally occurring hormone), blocks the action of VIP.[*[citation needed](https://en.wikipedia.org/wiki/Wikipedia:Citation_needed)*]

The best chance for a cure is surgery to remove the tumor. If the tumor has not spread to other organs, surgery can often achieve this.[*[citation needed](https://en.wikipedia.org/wiki/Wikipedia:Citation_needed)*]

For metastatic disease, [peptide receptor radionuclide therapy](/source/Peptide_receptor_radionuclide_therapy) (PRRT) can be highly effective. This treatment involves attaching a radionuclide (Lutetium-177 or Yttrium-90) to a somatostatin analogue (octreotate or octreotide). This is a novel way to deliver high doses of beta radiation to eradicate tumours. Some patients seem to respond to a combination chemotherapy consisting of capecitabine and temozolomide, but there is no report stating its curative effects.[*[citation needed](https://en.wikipedia.org/wiki/Wikipedia:Citation_needed)*]

## Prognosis

Surgery can usually serve as the cure. However, in one-third to one-half of patients, the tumor has metastasized to other organs by the time of diagnosis and cannot be cured, only treated.[*[citation needed](https://en.wikipedia.org/wiki/Wikipedia:Citation_needed)*]

## References

1. **[^](#cite_ref-1)** ["VIPoma"](https://web.archive.org/web/20090628224336/http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/nine/000116307.htm) at *[Dorland's Medical Dictionary](/source/Dorland's_medical_reference_works)*

1. **[^](#cite_ref-2)** Mansour JC, Chen H (Jul 2004). "Pancreatic endocrine tumors". *J Surg Res*. **120** (1): 139–61. [doi](/source/Doi_(identifier)):[10.1016/j.jss.2003.12.007](https://doi.org/10.1016%2Fj.jss.2003.12.007). [PMID](/source/PMID_(identifier)) [15172200](https://pubmed.ncbi.nlm.nih.gov/15172200).

1. **[^](#cite_ref-3)** ["VIPoma | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program"](https://web.archive.org/web/20180418032119/https://rarediseases.info.nih.gov/diseases/5493/vipoma). *rarediseases.info.nih.gov*. Archived from [the original](https://rarediseases.info.nih.gov/diseases/5493/vipoma) on 2018-04-18. Retrieved 2018-04-17.

1. **[^](#cite_ref-4)** Verner JV, Morrison AB (Sep 1958). "Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia". *Am J Med*. **25** (3): 374–80. [doi](/source/Doi_(identifier)):[10.1016/0002-9343(58)90075-5](https://doi.org/10.1016%2F0002-9343%2858%2990075-5). [PMID](/source/PMID_(identifier)) [13571250](https://pubmed.ncbi.nlm.nih.gov/13571250).

1. **[^](#cite_ref-5)** ["Carcinoid Tumors and Syndrome"](https://www.lecturio.com/concepts/carcinoid-tumors-and-syndrome/). *The Lecturio Medical Concept Library*. Retrieved 5 July 2021.

1. ^ [***a***](#cite_ref-auto_6-0) [***b***](#cite_ref-auto_6-1) Sandhu S, Jialal I (2024). ["ViPoma"](https://www.ncbi.nlm.nih.gov/books/NBK507698/). *National Center for Biotechnology Information, U.S. National Library of Medicine*. [PMID](/source/PMID_(identifier)) [29939520](https://pubmed.ncbi.nlm.nih.gov/29939520). Retrieved 5 July 2021.

- Jensen, R.T.; Norton, J.A. (2010). ["32. Endocrine tumors of the pancreas and gastrointestinal tract §VIPomas"](https://books.google.com/books?id=zEZOqB6r9hwC&pg=PA508&dq=%22VIPomas%22). In Feldman, M.; Friedman, L.S.; Brandt, L.J. (eds.). *Sleisenger and Fordtran's Gastrointestinal and Liver Disease* (9th ed.). Saunders Elsevier. pp. 508–511. [ISBN](/source/ISBN_(identifier)) [978-1-4377-2767-8](https://en.wikipedia.org/wiki/Special:BookSources/978-1-4377-2767-8).

- ["VIPoma"](https://web.archive.org/web/20130225102140/http://health.nytimes.com/health/guides/disease/vipoma/overview.html). *Health Guide*. New York Times. December 28, 2010. Archived from [the original](https://health.nytimes.com/health/guides/disease/vipoma/overview.html) on 2013-02-25.

## External links

Classification D ICD-10: C25.4 or E16.8 ICD-O: M8155/3 MeSH: D003969 DiseasesDB: 13877 SNOMED CT: 447643008 External resources MedlinePlus: 000228 eMedicine: med/2379 med/2399 ped/2428 Orphanet: 97282

v t e Glandular and epithelial cancer Epithelium Papilloma/carcinoma Small-cell carcinoma Combined small-cell lung carcinoma Verrucous carcinoma Squamous-cell carcinoma Basal-cell carcinoma Transitional cell carcinoma Inverted papilloma Complex epithelial Warthin's tumor Thymoma Bartholin gland carcinoma Glands Adenomas/ adenocarcinomas Gastrointestinal tract: Linitis plastica Familial adenomatous polyposis pancreas Insulinoma Glucagonoma Gastrinoma VIPoma Somatostatinoma Cholangiocarcinoma Klatskin tumor Hepatocellular adenoma/Hepatocellular carcinoma Urogenital Renal cell carcinoma Endometrioid tumor Renal oncocytoma Endocrine Prolactinoma Multiple endocrine neoplasia Adrenocortical adenoma/Adrenocortical carcinoma Hürthle cell Other/multiple Neuroendocrine tumor Carcinoid Adenoid cystic carcinoma Oncocytoma Clear-cell adenocarcinoma Apudoma Cylindroma Papillary hidradenoma Adnexal and skin appendage sweat gland Hidrocystoma Syringoma Syringocystadenoma papilliferum Cystic, mucinous, and serous Cystic general Cystadenoma/Cystadenocarcinoma Mucinous Signet ring cell carcinoma Krukenberg tumor Mucinous cystadenoma / Mucinous cystadenocarcinoma Pseudomyxoma peritonei Mucoepidermoid carcinoma Serous Ovarian serous cystadenoma / Pancreatic serous cystadenoma / Serous cystadenocarcinoma / Papillary serous cystadenocarcinoma Ductal, lobular, and medullary Ductal carcinoma Mammary ductal carcinoma Pancreatic ductal carcinoma Comedocarcinoma Paget's disease of the breast / Extramammary Paget's disease Lobular carcinoma Lobular carcinoma in situ Invasive lobular carcinoma Medullary carcinoma Medullary breast carcinoma Medullary thyroid cancer Acinar cell Acinic cell carcinoma

v t e Tumours of endocrine glands Pancreas Pancreatic cancer Pancreatic neuroendocrine tumor α: Glucagonoma β: Insulinoma δ: Somatostatinoma G: Gastrinoma VIPoma Pituitary Pituitary adenoma: Prolactinoma ACTH-secreting pituitary adenoma GH-secreting pituitary adenoma Craniopharyngioma Pituicytoma Thyroid Thyroid cancer (malignant): epithelial-cell carcinoma Papillary Follicular/Hurthle cell Parafollicular cell Medullary Anaplastic Lymphoma Squamous-cell carcinoma Benign Thyroid adenoma Struma ovarii Adrenal tumor Cortex Adrenocortical adenoma Adrenocortical carcinoma Medulla Pheochromocytoma Neuroblastoma Paraganglioma Parathyroid Parathyroid neoplasm Adenoma Carcinoma Pineal gland Pinealoma Pinealoblastoma Pineocytoma MEN 1 2A 2B

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Adapted from the Wikipedia article [VIPoma](https://en.wikipedia.org/wiki/VIPoma) by Wikipedia contributors ([contributor history](https://en.wikipedia.org/wiki/VIPoma?action=history)). Available under [Creative Commons Attribution-ShareAlike 4.0 International](https://creativecommons.org/licenses/by-sa/4.0/). Changes may have been made.