What is Zollinger-Ellison Syndrome (Gastrinoma)

In Zollinger-Ellison syndrome, the primary tumour is located in the pancreas. This type of tumour, however, secretes large quantities of a hormone called “gastrin” which causes the stomach to secrete much more acid than usual – leading to ulcers throughout the gatro-intestinal tract – in the stomach, duodenum or jejunum.


Anatomy of the gastrointestinal system

Statistics on Zollinger-Ellison Syndrome (Gastrinoma)

Precise data on the incidence is not available. Zollinger-Ellison syndrome is responsible for a small proportion of peptic ulcers.

Risk Factors for Zollinger-Ellison Syndrome (Gastrinoma)

This type of tumour (gastrinoma) is usually idiopathic. However, 20% of patients with Zollinger-Ellison syndrome have multiple endocrine neoplasia type 1 syndrome, characterised by other endocrine tumours.

Progression of Zollinger-Ellison Syndrome (Gastrinoma)

Medical treatment can completely treat the symptoms due to the ulcers.

These tumours are slow growing and generally cause no symptoms for many years, even decades. However, they are not always surgically removable and thus can be fatal, albeit after many years.

How is Zollinger-Ellison Syndrome (Gastrinoma) Diagnosed?

Full blood count – may indicate iron deficiency anaemia (consider iron studies).

Prognosis of Zollinger-Ellison Syndrome (Gastrinoma)

Patients may experience morbidity due to the ulcers, which can be complicated by massive bleeding or even perforation. The ulcers themselves are easily treated medically.

The tumour itself is a slow growing one as mentioned previously, but it is still malignant, and if not surgically removed has the potential to be fatal – although this may take many years. Unfortunately, up to 50% of patients may not be suitable for surgery due to spread of the tumour (e.g. to the liver or other surrounding organs or structures).

How is Zollinger-Ellison Syndrome (Gastrinoma) Treated?

Zollinger-Ellison syndromeThe manifestations of acid-peptic disease can be controlled with anti-ulcer medications. The treatment of choice is firstly, a proton pump inhibitor or secondarily, an H2 antagonist. The dose should be adjusted based on the effect and the basal acid outlet.

  •  Surgical approaches should only be considered in patients who are unable to tolerate medication.
  • A careful endoscopic exam – looking for ulcers.
  • Transhepatic portal venous sampling – mixed results.
  • Laparotamy – to remove the gastrinoma.

In those with advanced liver disease, a liver transplant can provide remarkable relief.

Zollinger-Ellison Syndrome (Gastrinoma) References

  1. Braunwald E, Fauci AS, Kasper DL, et al. Harrison’s Principles of Internal Medicine (15th edition). New York: McGraw-Hill Publishing; 2001.
  2. Cotran RS, Kumar V, Collins T, Robbins SL. Robbins Pathologic Basis of Disease (6th edition). Philadelphia: WB Saunders Company; 1999.
  3. Davidson S, Haslett C. Davidson’s Principles and Practice of Medicine (19th edition). Edinburgh: Churchill Livingstone; 2002.
  4. Hurst JW (ed). Medicine for the Practicing Physician (4th edition). Norwalk, CT. Appleton and Lange; 1996.
  5. Kumar P, Clark M. Clinical Medicine (5th edition). Edinburgh: WB Saunders; 2002.
  6. Longmore M, Wilkinson IB. Oxford Handbook of Clinical Medicine (5th edition). Oxford: Oxford University Press; 2001.
  7. McLatchie GR, Leaper DJ (eds). Oxford Handbook of Clinical Surgery (2nd edition). Oxford: Oxford University Press; 2002.
  8. Stone C. Zollinger-Ellison syndrome[online]. Bethesda, MD: MedlinePlus. Available from: URL link
  9. Raftery AT. Churchill’s Pocketbook of Surgery. Edinburgh: Churchill Livingsone; 2001.
  10. Tjandra JJ, Clunie GJA, Thomas RJS (eds). Textbook of Surgery (2nd edition). Melbourne: Wiley-Blackwell; 2001.