{"entity":{"id":"vipoma","kind":"cancer","name":"VIPoma","aka":["Vasoactive intestinal peptide-secreting tumour","Verner-Morrison syndrome","Pancreatic cholera","WDHA syndrome (watery diarrhoea, hypokalaemia, achlorhydria)"],"tldr":"VIPoma is a very rare pancreatic neuroendocrine tumour that secretes vasoactive intestinal peptide, causing litres of watery diarrhoea a day with dangerous loss of potassium. Most have spread to the liver by diagnosis. Fluid replacement and somatostatin analogues control the diarrhoea, surgery cures the few caught early, and the usual neuroendocrine tumour treatments are used for spread.","summary":"VIPoma is a functioning pancreatic neuroendocrine tumour secreting vasoactive intestinal peptide, producing the Verner-Morrison syndrome of watery diarrhoea, hypokalaemia and achlorhydria (Pancreatology 2021). In the French GTE series of 22 patients with strictly confirmed diagnoses, tumours were mostly metastatic (77 percent) and grade 2 (83 percent), median follow-up was 78.2 months, surgical excision of non-metastatic tumours controlled the secretory syndrome, and the antisecretory (over 50 percent fall in bowel movements) and antitumour efficacy of each treatment received was recorded (Pancreatology 2021).\n\nHow it differs from its parent: the emergency is metabolic rather than oncological; secretory diarrhoea persists during fasting and can cause renal failure and arrhythmia, so rehydration and octreotide come before any tumour-directed treatment.\n\nHow common: no registry figure in the sources read; the functioning tumours are a minority of pancreatic neuroendocrine tumours.\n\nTreatment: intravenous fluids and potassium; somatostatin analogues for the syndrome; resection for localised disease; the parent page's pathways (everolimus, sunitinib, peptide receptor radionuclide therapy, liver-directed therapy, chemotherapy for grade 3) for metastatic disease, with the GTE series as the evidence on antisecretory efficacy (Pancreatology 2021).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/VIPoma","links":[{"label":"NCI PDQ: pancreatic neuroendocrine (islet cell) tumour treatment","url":"https://www.cancer.gov/types/pancreatic/neuroendocrine-tumors/treatment"},{"label":"Pancreatology 2021: efficacy of treatments for VIPoma, French GTE multicentre series of 22 patients","url":"https://doi.org/10.1016/j.pan.2021.08.001"}],"tags":["subtype-page","wave4","rare"],"related":["pancreatic-net","glucagonoma","somatostatinoma","men1-syndrome","neuroendocrine"],"cancers":[],"sections":[],"technologies":["prrt"],"targets":[],"drugs":["everolimus","sunitinib"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"Very rare and life-threatening: seven French expert centres treated 22 patients between 1990 and 2016, 77 percent with metastatic disease and 83 percent grade 2 (Pancreatology 2021). No registry figure is in the sources read.","subtypes":["Pancreatic VIPoma, a functioning pancreatic neuroendocrine tumour (the usual site in adults)","Extrapancreatic VIP-secreting tumours (neurogenic tumours in children)","MEN1-associated VIPoma"],"biomarkers":["Plasma vasoactive intestinal peptide","Serum potassium and bicarbonate; stool volume","Chromogranin A; WHO grade (Ki-67)","Somatostatin receptor imaging"],"standardOfCare":[{"setting":"All cases","approach":"Rehydration and potassium; somatostatin analogues for the syndrome; resection when localised; the parent page's pathways for metastatic disease.","refs":["pancreatic-net","everolimus","sunitinib","prrt"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pancreatic-net"},"route":"/cancers/vipoma/","neighbours":{"cancer":[{"id":"glucagonoma","kind":"cancer","name":"Glucagonoma","route":"/cancers/glucagonoma/"},{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/"},{"id":"neuroendocrine","kind":"cancer","name":"Neuroendocrine tumours","route":"/cancers/neuroendocrine/"},{"id":"pancreatic-net","kind":"cancer","name":"Pancreatic neuroendocrine tumours","route":"/cancers/pancreatic-net/"},{"id":"somatostatinoma","kind":"cancer","name":"Somatostatinoma","route":"/cancers/somatostatinoma/"}],"technology":[{"id":"prrt","kind":"technology","name":"Peptide receptor radionuclide therapy (PRRT)","route":"/technologies/prrt/"}],"drug":[{"id":"everolimus","kind":"drug","name":"Everolimus","route":"/drugs/everolimus/"},{"id":"sunitinib","kind":"drug","name":"Sunitinib","route":"/drugs/sunitinib/"}]}}