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WHO Classification of Tumours
Vipoma, malignant
Pancreas


Definition

A VIPoma is a functionally active and usually malignant endocrine tumour, arising mostly in the pancreas, which secretes mainly vasoactive intestinal peptide (VIP). VIP-secreting tumours have been associated with the watery diarrhoea syndrome. Additional substances produced by VIPomas, possibly contributing to the clinical syndrome, include peptide histidine methionine (PHM), pancreatic polypeptide (PP), neurotensin, and others
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Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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Pancreatic VIPomas constitute about 80% of diarrheogenic neoplasms in the pancreas

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Klöppel G, Heitz PU (1988)
Pancreatic endocrine tumors.
Pathol Res Pract 183: 155-68



, half of them are malignant at the time of diagnosis. Females are affected more often than males. VIPomas are not familial, except for some tumours associated with MEN 1
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Hutcheon DF, Bayless TM, Cameron JL, Baylin SB (1979)
Hormone-mediated watery diarrhea in a family with multiple endocrine neoplasms.
Ann Intern Med 90: 932-4




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Ooi A, Kameya T, Tsumuraya M, Yamaguchi K, Abe K, Shimosato Y, Yanaihara N (1985)
Pancreatic endocrine tumours associated with WDHA syndrome. An immunohistochemical and electron microscopic study.
Virchows Arch A Pathol Anat Histopathol 405: 311-23



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After treatment, a meta-analysis study reported a 59.6% 5-year survival for patients with metastases and 94.4% for patients without metastases

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Soga J, Yakuwa Y (1998)
Vipoma/diarrheogenic syndrome: a statistical evaluation of 241 reported cases.
J Exp Clin Cancer Res 17: 389-400



. The massive diarrhoea with ensuing electrolyte imbalance associated with these tumours may initially pose a higher threat to the patient’s life than the growth and spread of the tumour itself.

Commentary: Neuroendocrine tumours of the digestive system