Zollinger-Ellison Syndrome (Gastrinoma)

What is Zollinger-Ellison Syndrome?

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, duedenum or jejunum.

Statistics on Zollinger-Ellison Syndrome?

Precise data on the incidence is not available. ZES is responsible for a small proportion of peptic ulcers.

Risk Factors for Zollinger-Ellison Syndrome

This type of tumour (gastrinoma) is usually idiopathic. However, 20% of patients with ZES have the Multiple Endocrine Neoplasia type 1 syndrome - characterised by other endocrine tumours.

Progression of Zollinger-Ellison Syndrome

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 Diagnosed?

Full blood count - may indicate iron deficiency anaemia (Consider iron studies)

Prognosis of Zollinger-Ellison Syndrome

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 Treated?

The 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 References

Braunwald, Fauci, Kasper, Hauser, Longo, Jameson. Harrison's Principles of Internal Medicine. 15th Edition. McGraw-Hill. 2001
Cotran, Kumar, Collins 6th edition. Robbins Pathologic Basis of Disease. WB Saunders Company. 1999.
Haslet C, Chiliers ER, Boon NA, Colledge NR. Principles and Practice of Medicine. Churchill Livingstone 2002.
Hurst JW (Editor-in-chief). Medicine for the practicing physician. 4th edition Appleton and Lange 1996.
Kumar P, Clark M. CLINICAL MEDICINE. WB Saunders 2002
Longmore M, Wilkinson I, Torok E. OXFORD HANDBOOK OF CLINICAL MEDICINE. Oxford Universtiy Press. 2001
McLatchie G and LEaper DJ (editors). Oxford Handbook of Clinical Surgery 2nd Edition. Oxford University Press 2002.
MEDLINE Plus
Raftery AT Churchill's pocketbook of Surgery. Churchill Livingsone 2001.
Tjandra, JJ, Clunie GJ, Thomas, RJS,; Textbook of Surgery, 2nd Ed, Blackwell Science, Asia. 2001.