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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.
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).
How 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.
How common: no registry figure in the sources read; the functioning tumours are a minority of pancreatic neuroendocrine tumours.
Treatment: 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).
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.
Most pancreatic cancers arise in the head next to the bile duct, which is why jaundice is the presenting sign; bile duct cancers are named by where along the tree they sit.
Same organ: Glucagonoma, Somatostatinoma, Pancreatic ductal adenocarcinoma, Biliary tract cancer (cholangiocarcinoma), Intrahepatic cholangiocarcinoma, Extrahepatic cholangiocarcinoma (perihilar and distal), Biliary tract cancer (all types), Neuroendocrine tumours, Pancreatic neuroendocrine tumours, Grade 3 well-differentiated neuroendocrine tumour, Extrapulmonary neuroendocrine carcinoma, Gallbladder cancer, Gallbladder adenocarcinoma, Papillary carcinoma of the gallbladder, Mucinous carcinoma of the gallbladder, Adenosquamous and squamous carcinoma of the gallbladder, Neuroendocrine carcinoma of the gallbladder, Incidental gallbladder cancer (found after cholecystectomy), Carcinoma in situ and dysplasia of the gallbladder, Cystic duct carcinoma, Ampullary cancer (ampulla of Vater), Resectable pancreatic ductal adenocarcinoma, Borderline resectable pancreatic ductal adenocarcinoma, Locally advanced unresectable pancreatic ductal adenocarcinoma, Metastatic pancreatic ductal adenocarcinoma, KRAS G12C-mutant pancreatic ductal adenocarcinoma, KRAS wild-type pancreatic ductal adenocarcinoma, BRCA or PALB2-mutant pancreatic ductal adenocarcinoma, Mismatch repair deficient (MSI-high) pancreatic ductal adenocarcinoma, Pancreatic acinar cell carcinoma, Intraductal papillary mucinous neoplasm and other pancreatic cystic precursors, Pancreatoblastoma
Rehydration and potassium; somatostatin analogues for the syndrome; resection when localised; the parent page's pathways for metastatic disease.
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Query for this cancer: (TITLE:"VIPoma" OR ABSTRACT:"VIPoma" OR TITLE:"Vasoactive intestinal peptide-secreting tumour" OR ABSTRACT:"Vasoactive intestinal peptide-secreting tumour" OR TITLE:"Verner-Morrison syndrome" OR ABSTRACT:"Verner-Morrison syndrome" OR TITLE:"Pancreatic cholera" OR ABSTRACT:"Pancreatic cholera" OR TITLE:"WDHA syndrome watery diarrhoea, hypokalaemia, achlorhydria" OR ABSTRACT:"WDHA syndrome watery diarrhoea, hypokalaemia, achlorhydria") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about VIPoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Avoid grapefruit. Live vaccines are contraindicated.
Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
7.5 mg (mild), 5 mg (moderate), 2.5 mg (severe).
See all on the product pages:EverolimusSunitinib·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with VIPoma, then print the one-page appointment sheet with room for the answers.
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