Everything about Vipoma totally explained
A
VIPoma (also known as
Verner Morrison syndrome, after the physicians who first described it ) 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).
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
watery
diarrhea and resultant
dehydration,
hypokalemia,
achlorhydria (hence
WDHA-syndrome, or
pancreatic cholera syndrome), acidosis,
vasodilation (
flushing and
hypotension),
hypercalcemia and
hyperglycemia.
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
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.
Treatment
Besides treating the
water and
electrolyte abnormalities,
octreotide (a
somatostatin analogue) can be used to temper symptoms.
Surgery is the only curative option.
Further Information
Get more info on 'Vipoma'.
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