Disease Directory VIPoma
Endocrine

VIPoma

Also known as: Verner-Morrison syndrome, pancreatic cholera, WDHA syndrome, vasoactive intestinal peptide tumor

Prevalence

Less than 1 per million per year

Onset

Most commonly in adults aged 40–60

Type

Sporadic (majority); rarely associated with MEN1

About VIPoma

VIPoma is a rare pancreatic or extra-pancreatic neuroendocrine tumour that secretes excess vasoactive intestinal peptide (VIP), causing a distinctive syndrome of profuse watery diarrhoea, hypokalaemia, and achlorhydria collectively termed WDHA syndrome or Verner-Morrison syndrome. The massive secretory diarrhoea, which persists even with fasting, leads to severe electrolyte disturbances, dehydration, and metabolic acidosis that can be life-threatening if untreated. Over 50% of VIPomas are malignant at the time of diagnosis, with liver metastases commonly present, making systemic therapy trials particularly relevant for this tumour type.

Common Clinical Features

Profuse watery diarrhoea (3–5 litres or more per day) Severe hypokalaemia Achlorhydria or hypochlorhydria Dehydration and weakness Flushing episodes Hyperglycaemia Hypercalcaemia Weight loss and malnutrition

Clinical Trial Eligibility Tips

What to know before applying to VIPoma trials.

Elevated fasting plasma VIP level (typically greater than 200 pg/mL) combined with characteristic symptoms and imaging evidence of a pancreatic or retroperitoneal mass is required for trial eligibility confirmation.

Correction of electrolyte abnormalities (particularly hypokalaemia) to safe levels before screening is a standard requirement; recent electrolyte results and repletion history should be documented.

Functional imaging with 68Ga-DOTATATE PET or octreotide scan assessing somatostatin receptor expression is required for trials of somatostatin analogues or PRRT; receptor positivity is often an eligibility criterion.

Patient Resources

Patient Organization

Neuroendocrine Tumor Research Foundation

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Natural History Registry

NANETS Patient Registry

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Orphanet

European reference resource for rare diseases (ORPHA:525)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting VIPoma trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for VIPoma. Updated daily.

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