VIPomas are a very rare pancreatic endocrine tumour that classically presents with watery diarrhoea and hypokalaemia. These tumours secrete, and get their name from, vasoactive intestinal peptide (VIP).
VIPomas represent <2% of pancreatic endocrine tumours 2.
Patients typically present with watery diarrhoea, hypokalaemia, and achlorhydria, which has been given the acronym WDHA 1.
VIPomas are usually large (~5 cm) at presentation with most patient (~70%) having metastatic disease 3.
- intrapancreatic (~75%): most commonly in the pancreatic tail
- extrapancreatic neurogenic (~20%): arising from the sympathetic chain
- extrapancreatic non-neurogenic (~5%): arising in the oesophageal, bowel, liver, and kidney 3
- MEN 1 (rare) 3
- 1. Vinik A. Vasoactive Intestinal Peptide Tumor (VIPoma) [Updated 2013 Nov 28]. In: De Groot LJ, Beck-Peccoz P, Chrousos G, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: http://www.ncbi.nlm.nih.gov/books/NBK278960/
- 2. Camera L, Severino R, Faggiano A et-al. Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma. World J Radiol. 2014;6 (10): 840-5. doi:10.4329/wjr.v6.i10.840 - Free text at pubmed - Pubmed citation
- 3. Lewis RB, Lattin GE, Paal E. Pancreatic endocrine tumors: radiologic-clinicopathologic correlation. Radiographics. 2010;30 (6): 1445-64. doi:10.1148/rg.306105523 - Pubmed citation