Zollinger Ellison Syndrome
Also known as Gastrinoma, ZES
This article is general health information for the selected condition. It is not a diagnosis and does not replace clinical judgement. If you have questions or need treatment advice, please consult a qualified healthcare professional.
What is zollinger ellison syndrome?
Zollinger Ellison syndrome is caused by a gastrin secreting tumour, usually in the pancreas or duodenum, that drives massive stomach acid production. This produces severe, multiple or recurrent peptic ulcers that resist standard treatment, often with diarrhoea. It should be suspected when ulcers recur despite H pylori eradication and acid suppression.
Causes
- Gastrin secreting neuroendocrine tumour
- Sporadic tumours in the majority of cases
- Association with multiple endocrine neoplasia type 1
- Excess gastrin driving parietal cell acid secretion
Symptoms
- Severe or recurrent peptic ulcers
- Burning upper abdominal pain
- Ulcers in unusual locations beyond the duodenal bulb
- Chronic diarrhoea and fatty stools
- Heartburn resistant to standard treatment
- Nausea and vomiting
- Weight loss
- Gastrointestinal bleeding
Risk factors
- Multiple endocrine neoplasia type 1
- Family history of endocrine tumours
- Age typically 20 to 60 at diagnosis
- Male sex slightly more common
Diagnosis
- Fasting serum gastrin measured off proton pump inhibitors
- Gastric pH testing to confirm acid hypersecretion
- Secretin stimulation test
- Endoscopic ultrasound and cross sectional imaging
- Somatostatin receptor imaging to localise tumours
- Screening for multiple endocrine neoplasia type 1
Treatment
- High dose proton pump inhibitor therapy to control acid
- Surgical resection of the gastrinoma where localised
- Somatostatin analogues for advanced disease
- Targeted therapy or chemotherapy for metastatic tumours
- Lifelong monitoring for recurrence
- Family screening where MEN1 is confirmed
Self care
- Take high dose acid suppression exactly as prescribed and never stop suddenly
- Avoid anti inflammatory painkillers
- Eat smaller regular meals
- Avoid alcohol and stop smoking
- Attend all surveillance imaging appointments
- Carry details of your diagnosis, since acid suppression must not be interrupted
- Watch for symptoms of low vitamin B12 on long term acid suppression
When to see a doctor
Book an appointment if:
- Ulcers recurring despite treatment and H pylori eradication
- Chronic diarrhoea with reflux or ulcer symptoms
- Ulcers in unusual locations
- Family history of endocrine tumours
- Symptoms of high calcium such as thirst and confusion
- Weight loss with ongoing abdominal pain
Some symptoms need emergency care rather than an appointment. See the warning signs listed above.
Prevention
- The syndrome cannot be prevented
- Complications are prevented by continuous acid suppression
- Screen relatives where multiple endocrine neoplasia type 1 is confirmed
- Regular surveillance detects tumour progression early
- Never interrupt proton pump inhibitor therapy without specialist advice
Outlook
Acid related symptoms are well controlled by high dose proton pump inhibitors in almost everyone. Long term outlook depends on the tumour itself: surgically removable gastrinomas can be cured, while metastatic disease is typically slow growing with reasonable long term survival. Ten year survival is good where liver metastases are absent.
Common questions
When should this rare condition be suspected?
Consider it with multiple ulcers, ulcers beyond the duodenal bulb, ulcers recurring despite H pylori eradication and acid suppression, ulcers with chronic diarrhoea, or a family history of endocrine tumours.
Why must acid suppression be stopped before testing?
Proton pump inhibitors raise gastrin levels on their own and produce false positive results. They usually need withdrawing under supervision before gastrin is measured, which requires careful planning.
Are these tumours cancerous?
Many are malignant but typically slow growing, and around half have spread by diagnosis. Long term survival is often good with acid control and tumour directed treatment, particularly when resection is possible.
References
Important: Dard AI is not a medical device and does not diagnose, treat, or replace clinician judgement. This article is general information only. Always consult a qualified healthcare professional about diagnosis and treatment, and seek emergency care for severe or rapidly worsening symptoms.