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   Image          = Pancreatic vipoma.jpg|
   Image          = Pancreatic vipoma.jpg|
   Caption        = Pancreatic vipoma. Electron microscopy of a pancreatic VIPoma. Abundant secretory granules of variable size, shape, and density in a pancreatic tumor with WDHA syndrome. Abundant PP-and a few VIP-immunoreactive cells (inset) were detected by light microscopic immunohistochemistry of the same tumor (X28.000). <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
   Caption        = Pancreatic vipoma. Electron microscopy of a pancreatic VIPoma. Abundant secretory granules of variable size, shape, and density in a pancreatic tumor with WDHA syndrome. Abundant PP-and a few VIP-immunoreactive cells (inset) were detected by light microscopic immunohistochemistry of the same tumor (X28.000). <br> <small> [http://www.peir.net Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology] </small>|
  DiseasesDB    = 13877 |
 
  ICD10          = {{ICD10|C|25|4|c|15}} or {{ICD10|E|16|8|e|15}} |
  ICD9          = |
  ICDO          = 8155/3 |
  OMIM          = |
  MedlinePlus    = 000228 |
  MeshID        = D003969 |
}}
}}
'''For patient information, click [[Jaundice (patient information)|here]]'''
'''For patient information, click [[VIPoma (patient information)|here]]'''
{{SI}}
{{VIPoma}}
{{CMG}}
{{CMG}}; {{AE}}{{Homa}}{{MSI}} {{PSD}}


{{SK}} Watery diarrhea with hypokalemic alkalosis; WDHA syndrome; pancreatic cholera syndrome; Verner-Morrison syndrome.
{{SK}} [[VIPomas]][[Watery diarrhea with hypokalemic alkalosis|; watery diarrhea with hypokalemic alkalosis]]; [[WDHA syndrome]]; [[watery diarrhea hypokalemia achlorhydria syndrome]]; [[pancreatic cholera syndrome]]; [[Verner-Morrison syndrome|Verner-Morrison syndrome;]] [[non-beta pancreatic islet cell tumor]]; [[diarrheogenic islet cell tumor]]; [[VIP-secreting tumor]]; [[vasoactive intestinal polypeptide secreting tumor]], [[pancreatic VIPoma]]


==Overview==
==[[VIPoma overview|Overview]]==


A '''VIPoma''' (also known as '''[[Verner Morrison syndrome]]''', after the physicians who first described it <ref>Verner, J. V., and Morrison, A. B. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. ''Am J Med'' 1958; '''374''': 1958.</ref>) is a rare (1 per 10'000'000 per year) [[endocrine]] [[tumor]], usually (about 90%) originating in the [[pancreas]], which produces [[vasoactive intestinal peptide]] (VIP).
==[[VIPoma historical perspective|Historical Perspective]]==


* A syndrome caused by non-β islet-cell tumors.
==[[VIPoma pathophysiology|Pathophysiology]]==
* It may be associated with multiple endocrine neoplasia.


The massive amounts of VIP in turn cause profound and chronic '''w'''atery '''d'''[[diarrhea|iarrhea]] and resultant  [[dehydration]], '''h'''[[hypokalemia|ypokalemia]], '''a'''[[achlorhydria|chlorhydria]] (hence '''WDHA-syndrome''', or '''pancreatic cholera syndrome'''), acidosis, [[vasodilation]] ([[flushing (physiology)|flushing]] and [[hypotension]]), [[hypercalcemia]] and [[hyperglycemia]].<ref>Mansour JC, Chen H. Pancreatic endocrine tumors. ''J Surg Res'' 2004; '''120''': 139-61. PMID 15172200</ref>
==[[VIPoma causes|Causes]]==


==Symptoms and Signs==
==[[VIPoma differential diagnosis|Differentiating VIPoma from other Diseases]]==
The Major clinical features are prolonged watery [[diarrhea]] (fasting stool volume > 750 to 1000 mL/day)and symptoms of [[hypokalemia]] and [[dehydration]].
Half of the patients have relatively constant [[diarrhea]] while the rest have alternating periods of severe and moderate [[diarrhea]].
One third have diarrhea < 1yr before diagnosis ,but in 25%, diarrhea is present for 5 yr or more before diagnosis.
[[Lethargy]], muscle weakness, [[nausea]], vomiting and crampy abdominal pain are frequent symptoms.
[[Hyperkalemia]] and impaired [[glucose]] tolerance occur in < 50% of patients.
During attacks of diarrhea, flushing similar to the [[carcinoid syndrome]] occur rarely.


==Diagnosis==
==[[VIPoma epidemiology and demographics|Epidemiology and Demographics]]==
Besides the clinical picture, [[fasting]] VIP [[blood plasma|plasma]] dosage may confirm the diagnosis, and [[CT scan]] and [[somatostatin]] receptor [[scintigraphy]] are used to localise the [[tumor]], which is usually [[metastasis|metastatic]] at presentation.
 
==[[VIPoma risk factors|Risk Factors]]==
 
==[[VIPoma screening|Screening]]==


==Treatment==
==[[VIPoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
Besides treating the [[water]] and [[electrolyte]] abnormalities, [[octreotide]] (a [[somatostatin]] analogue) can be used to temper symptoms. [[Surgery]] is the only curative option.


==References==
==Diagnosis==
<references/>


==Additional Resources==
[[VIPoma history and symptoms|History and Symptoms]] | [[VIPoma physical examination|Physical Examination]] | [[VIPoma laboratory findings|Laboratory Findings]] | [[VIPoma x ray|X Ray]] | [[VIPoma CT|CT]] | [[VIPoma MRI|MRI]] | [[VIPoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[VIPoma other imaging findings|Other Imaging Findings]] |
[[VIPoma other diagnostic studies|Other Diagnostic Studies]]


^The MERCK MANUAL Of Diagnosis And Therapy
==Treatment==


<br>
[[VIPoma medical therapy|Medical Therapy]] | [[VIPoma surgery|Surgery]] | [[VIPoma primary prevention|Primary Prevention]] | [[VIPoma secondary prevention|Secondary Prevention]] | [[VIPoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[VIPoma future or investigational therapies|Future or Investigational Therapies]]


[[Category:Endocrinology]]
==Case Studies==
[[Category:Types of cancer]]


[[de:Verner-Morrison-Syndrom]]
[[VIPoma case study one|Case #1]]
[[pl:Guz wydzielający VIP]]






{{Tumor morphology}}


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Latest revision as of 18:07, 23 September 2023

VIPoma
Pancreatic vipoma. Electron microscopy of a pancreatic VIPoma. Abundant secretory granules of variable size, shape, and density in a pancreatic tumor with WDHA syndrome. Abundant PP-and a few VIP-immunoreactive cells (inset) were detected by light microscopic immunohistochemistry of the same tumor (X28.000).
Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Homa Najafi, M.D.[2]Madhu Sigdel M.B.B.S.[3] Parminder Dhingra, M.D. [4]

Synonyms and keywords: VIPomas; watery diarrhea with hypokalemic alkalosis; WDHA syndrome; watery diarrhea hypokalemia achlorhydria syndrome; pancreatic cholera syndrome; Verner-Morrison syndrome; non-beta pancreatic islet cell tumor; diarrheogenic islet cell tumor; VIP-secreting tumor; vasoactive intestinal polypeptide secreting tumor, pancreatic VIPoma

Overview

Historical Perspective

Pathophysiology

Causes

Differentiating VIPoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1



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