VIPomas
Pancreatic endocrine tumors associated with profound diarrhea, hypokalemia,
and achlorhydira were first described by Verner and Morrison in 1958. This syndrome
was subsequently found to be due to ectopic secretion of vasoactive intestinal
peptide (VIP) VIPomas most often present in the 5th decade of life and have
a similar incidence in men and women. The diagnosis is generally based on the
presence of elevated serum VIP levels, symptoms of diarrhea, and documentation
of malignancy. Somatostatin analogs are highly effective in suppressing hormone
secretion and in controlling the secretory diarrhea associated with the VIPoma
syndrome. Surgical resection is generally undertaken with curative intent in
patients with localized disease or for the purpose of cytoreduction in patients
with metastases.
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