VIPoma
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| ICD-10 | C25.4 or E16.8 |
|---|---|
| ICD-O: | 8155/3 |
| DiseasesDB | 13877 |
| MedlinePlus | 000228 |
| eMedicine | med/2379 med/2399 ped/2428 |
| MeSH | D003969 |
A VIPoma (also known as Werner Morrison syndrome, after the physicians who first described it [1]) 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).
The massive amounts of VIP in turn cause profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria (hence WDHA-syndrome, or pancreatic cholera syndrome), vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.[2]
[edit] Diagnosis
Besides the clinical picture, fasting VIP plasma dosage may confirm the diagnosis, and CT scan and somatostatin receptor scintigraphy are used to localise the tumor, which is usually metastatic at presentation.
[edit] Treatment
Besides treating the water and electrolyte abnormalities, octreotide (a somatostatin analogue) can be used to temper symptoms. Surgery is the only curative option.
[edit] References
- ^ 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.
- ^ Mansour JC, Chen H. Pancreatic endocrine tumors. J Surg Res 2004; 120: 139-61. PMID 15172200
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