Dr Rohit Sharma and Dr Henry Knipe et al.

VIPomas are a very rare type of pancreatic endocrine tumour that secrete, and get their name from, vasoactive intestinal peptide (VIP). The clinical syndrome resulting from these tumours is commonly known as WDHA syndrome, as an acronym of the cardinal symptoms of watery diarrhoea, hypokalaemia, and achlorhydria. 

VIPomas represent <2% of pancreatic endocrine tumours 2. There are two epidemiological peaks in the diagnosis of these tumours, one peak in middle-age adults and another peak in children aged between two and four years 1.

Patients present with the classic clinical triad of 1:

  • watery diarrhoea that persists with fasting
  • hypokalaemia
  • achlorhydria or hypochlorhydria

VIP has many roles in the body, however importantly is involved in secreting fluid and electrolytes into the lumen, inhibiting of gastric acid secretion, and stimulating glycogenolysis 1. Over-activity of these functions explain the classic symptoms of secretory diarrhoea and electrolyte deficiencies 1.

VIPomas are usually large (~5 cm) at presentation with most patients (~70%) having metastatic disease, especially involving the liver 3

  • intrapancreatic (~75%): most commonly in the pancreatic tail
  • extrapancreatic neurogenic (~20%): arising from the sympathetic chain
  • extrapancreatic non-neurogenic (~5%): arising in the oesophagous, bowel, liver, and kidney 3

VIPomas share the radiographic features of other pancreatic endocrine tumours. See pancreatic endocrine tumour for a detailed discussion regarding these features.

Acute management includes fluid and electrolyte replacement,as well as symptomatic management of diarrhoea with agents such as octreotide which inhibit VIP secretion 4. Once stable, surgical management is necessary, of both the primary tumour (e.g. distal pancreatomy) and metastases (e.g. hepatic resection) 5.

The tumour and clinical syndrome was first described by John V Verner, an American endocrinologist, and Ashton B Morrison, an American pathologist, in their 1958 seminal paper 6,7.

Pancreatic pathology
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Article information

rID: 42610
Synonyms or Alternate Spellings:
  • WDHA syndrome
  • Verner-Morrison syndrome

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