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
- 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
- MEN 1 (rare) 3
Treatment and prognosis
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.
History and etymology
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.
- 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
- 4. O'Dorisio TM, Mekhjian HS, Gaginella TS. Medical therapy of VIPomas. (1989) Endocrinology and metabolism clinics of North America. 18 (2): 545-56. Pubmed
- 5. Smith SL, Branton SA, Avino AJ, Martin JK, Klingler PJ, Thompson GB, Grant CS, van Heerden JA. Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. (1998) Surgery. 124 (6): 1050-5. Pubmed
- 6. Verner JV, Morrison AB. Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. (1958) The American journal of medicine. 25 (3): 374-80. Pubmed
- 7. Demeure MJ. John V. Verner Jr. and Ashton B. Morrison. InSurgical Endocrinopathies 2015 (pp. 313-315). Springer International Publishing.
- cystic neoplasm (cystic pancreatic mass differential diagnosis)
- solid neoplasm
- nonepithelial pancreatic neoplasms
pancreatitis (mnemonic for the causes)
- gallstone pancreatitis
- interstitial oedematous pancreatitis
- necrotising pancreatitis
- haemorrhagic pancreatitis
- revised Atlanta classification of acute pancreatitis
- chronic pancreatitis
- Ascaris-induced pancreatitis
- tropical pancreatitis
- autoimmune pancreatitis
- emphysematous pancreatitis
- hereditary pancreatitis
- pancreatitis associated with cystic fibrosis
- segmental pancreatitis
- acute pancreatitis
- pancreatic atrophy
- pancreatic lipomatosis
- pancreatic trauma