Definition
VIPoma is a functioning pancreatic neuroendocrine tumor producing the vasoactive intestinal peptide (VIP) and causing the Verner-Morrison or WDHA (watery diarrhea, hypokalemia, and achlorhydria) syndrome. However, it is worth noting that 10–25% of the WDHA syndrome is associated with extra-pancreatic neoplasms including neuroblastoma, ganglioneuroblastoma, ganglioneuroma, pheochromocytoma, composite pheochromocytoma, and paraganglioma. These cases are more often observed in pediatric population.
Clinical Features
Incidence
The estimated annual incidence of pancreatic VIPomas is 0.05–0.2 cases per 1 million people (Falconi et al. 2016). They account for 0.6–1.5% of all pancreatic neuroendocrine neoplasms and approximately 2–6% of all functioning pancreatic neuroendocrine tumors (PanNETs) (Ito et al. 2015).
Age
The average age of patients with pancreatic VIPomas at the time of diagnosis is 50.5 years (range of 15–82 years).
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References and Further Reading
Angelousi, A., Koffas, A., Grozinsky-Glasberg, S., Gertner, J., Kassi, E., Alexandraki, K., Caplin, M. E., Kaltsas, G., & Toumpanakis, C. (2019). Diagnostic and management challenges in vasoactive intestinal peptide secreting tumors: A series of 15 patients. Pancreas, 48, 934–942.
Barghorn, A., Komminoth, P., Bachmann, D., Rütimann, K., Saremaslani, P., Muletta-Feurer, S., Perren, A., Roth, J., Heitz, P. U., & Speel, E. J. (2001a). Deletion at 3p25.3-p23 is frequently encountered in endocrine pancreatic tumours and is associated with metastatic progression. The Journal of Pathology, 194, 451–458.
Barghorn, A., Speel, E. J., Farspour, B., Saremaslani, P., Schmid, S., Perren, A., Roth, J., Heitz, P. U., & Komminoth, P. (2001b). Putative tumor suppressor loci at 6q22 and 6q23-q24 are involved in the malignant progression of sporadic endocrine pancreatic tumors. The American Journal of Pathology, 158, 1903–1911.
Bartsch, D., Hahn, S. A., Danichevski, K. D., Ramaswamy, A., Bastian, D., Galehdari, H., Barth, P., Schmiegel, W., Simon, B., & Rothmund, M. (1999). Mutations of the DPC4/Smad4 gene in neuroendocrine pancreatic tumors. Oncogene, 18, 2367–2371.
Capella, C., & La Rosa, S. (2015). VIPoma. In S. La Rosa & F. Sessa (Eds.), Pancreatic neuroendocrine neoplasms. Practical approach to diagnosis, classification, and therapy (pp. 97–104). New York: Springer.
Falconi, M., Eriksson, B., Kaltsas, G., et al. (2016). ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology, 103, 153–171.
Ghaferi, A. A., Chojnacki, K. A., Long, W. D., Cameron, J. L., & Yeo, C. J. (2008). Pancreatic VIPomas: Subject review and one institutional experience. Journal of Gastrointestinal Surgery, 12, 382–893.
Ito, T., Igarashi, H., Nakamura, K., Sasano, H., Okusaka, T., Takano, K., Komoto, I., Tanaka, M., Imamura, M., Jensen, R. T., Takayanagi, R., & Shimatsu, A. (2015). Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: A nationwide survey analysis. Journal of Gastroenterology, 50, 58–64.
Müller, S., Kupka, S., Königsrainer, I., Northoff, H., Sotlar, K., Bock, T., Kandolf, R., Traub, F., Königsrainer, A., & Zieker, D. (2012). MSH2 and CXCR4 involvement in malignant VIPoma. World Journal of Surgical Oncology, 10, 264.
Siddappa, P. K., & Vege, S. S. (2019). Vasoactive intestinal peptide-secreting tumors: A review. Pancreas, 48, 1119–1125.
Smith, S. L., Branton, S. A., Avino, A. J., Martin, J. K., Klingler, P. J., Thompson, G. B., Grant, C. S., & van Heerden, J. A. (1998). Vasoactive intestinal polypeptide secreting islet cell tumors: A 15-year experience and review of the literature. Surgery, 124, 1050–1055.
Soga, J., & Yakuwa, Y. (1998). Vipoma/diarrheogenic syndrome: A statistical evaluation of 241 reported cases. Journal of Experimental & Clinical Cancer Research, 17, 389–400.
Solcia, E., Capella, C., Riva, C., Rindi, G., & Polak, J. M. (1988). The morphology and neuroendocrine profile of pancreatic epithelial VIPomas and extrapancreatic, VIP-producing, neurogenic tumors. Annals of the New York Academy of Sciences, 527, 508–517.
Speel, E. J., Richter, J., Moch, H., Egenter, C., Saremaslani, P., Rütimann, K., Zhao, J., Barghorn, A., Roth, J., Heitz, P. U., & Komminoth, P. (1999). Genetic differences in endocrine pancreatic tumor subtypes detected by comparative genomic hybridization. The American Journal of Pathology, 155, 1787–1794.
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La Rosa, S. (2022). VIPoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5203-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5203-1
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