VIPoma
3085380
226087194
2008-07-16T19:41:27Z
Arcadian
104523
ref
{{Infobox_Disease |
Name = VIPoma |
Image = |
Caption = |
DiseasesDB = 13877 |
ICD10 = {{ICD10|C|25|4|c|15}} or {{ICD10|E|16|8|e|15}} |
ICD9 = |
ICDO = 8155/3 |
OMIM = |
MedlinePlus = 000228 |
eMedicineSubj = med |
eMedicineTopic = 2379 |
eMedicine_mult = {{eMedicine2|med|2399}} {{eMedicine2|ped|2428}} |
MeshID = D003969 |
}}
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]],<ref name="urlDorlands Medical Dictionary:VIPoma">{{cite web |url=http://www.mercksource.com/pp/us/cns/cns_hl_dorlands_split.jsp?pg=/ppdocs/us/common/dorlands/dorland/nine/000116307.htm |title=Dorlands Medical Dictionary:VIPoma |format= |work= |accessdate=}}</ref> usually (about 90%) originating in the [[pancreas]], which produces [[vasoactive intestinal peptide]] (VIP).
A syndrome caused by [[non-β islet-cell]] tumors.
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>
==Symptoms and Signs==
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==
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.
==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==
<references/>
[[Category:Endocrinology]]
[[Category:Types of cancer]]
[[de:Verner-Morrison-Syndrom]]
[[pl:Guz wydzielający VIP]]
^The MERCK MANUAL Of Diagnosis And Therapy
{{oncology-stub}}
{{Tumor morphology}}