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{{SK}} Watery diarrhea with hypokalemic alkalosis; WDHA syndrome; pancreatic cholera syndrome; Verner-Morrison syndrome.
{{SK}} Watery diarrhea with hypokalemic alkalosis; WDHA syndrome; pancreatic cholera syndrome; Verner-Morrison syndrome.


==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]].
==[[VIPoma epidemiology and demographics|Epidemiology and Demographics]]==
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.
==[[VIPoma risk factors|Risk Factors]]==
[[Hyperkalemia]] and impaired [[glucose]] tolerance occur in < 50% of patients.
 
During attacks of diarrhea, flushing similar to the [[carcinoid syndrome]] occur rarely.
==[[VIPoma screening|Screening]]==
 
==[[VIPoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
==Diagnosis==
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 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]]


==Treatment==
==Treatment==
Besides treating the [[water]] and [[electrolyte]] abnormalities, [[octreotide]] (a [[somatostatin]] analogue) can be used to temper symptoms. [[Surgery]] is the only curative option.


==References==
[[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]]
<references/>


==Additional Resources==
==Case Studies==


^The MERCK MANUAL Of Diagnosis And Therapy
[[VIPoma case study one|Case #1]]


<br>


[[Category:Endocrinology]]
[[Category:Endocrinology]]
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Oncology]]


[[de:Verner-Morrison-Syndrom]]
[[de:Verner-Morrison-Syndrom]]

Revision as of 01:00, 19 September 2012

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
ICD-10 C25.4 or E16.8
ICD-O: 8155/3
DiseasesDB 13877
MedlinePlus 000228
MeSH D003969

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Watery diarrhea with hypokalemic alkalosis; WDHA syndrome; pancreatic cholera syndrome; Verner-Morrison syndrome.

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

de:Verner-Morrison-Syndrom


Template:Tumor morphology


Template:WikiDoc Sources