Medical conditionICD-10 E16.4

Zollinger-Ellison syndrome

A rare condition in which a tumour (a gastrinoma) produces the hormone gastrin uncontrollably, driving the stomach to make far too much acid. The result is severe, multiple or recurrent peptic ulcers and diarrhoea. It is diagnosed with a very high fasting gastrin alongside an acidic stomach.

What it is

Zollinger-Ellison syndrome is caused by a gastrinoma — a neuroendocrine tumour, usually in the pancreas or the wall of the duodenum, that secretes the hormone gastrin without the normal controls. Gastrin's job is to stimulate stomach acid, so a relentless excess drives the stomach to produce enormous amounts of acid. This acid overwhelms the protective lining of the stomach and duodenum, causing peptic ulcers that are typically severe, multiple, in unusual locations, or that keep coming back despite standard treatment. The acid load also causes diarrhoea (partly by inactivating digestive enzymes), and heartburn. A proportion of gastrinomas occur as part of an inherited syndrome (MEN1), so a family history and other hormone problems are looked for. Diagnosis rests on finding a markedly raised fasting blood gastrin while the stomach is still confirmed to be very acidic — the combination that separates it from the far commoner, harmless causes of a high gastrin (acid-suppressing medicines, low-acid states). Treatment is highly effective: powerful acid-suppressing medication controls the ulcers and symptoms, and the tumour itself is located with imaging and, where possible, removed. Recognising the pattern behind unusually stubborn ulcers is what leads to the diagnosis.

Key lab markers

  • Fasting gastrin — the key test; markedly raised, interpreted alongside stomach acidity.
  • Stomach acidity (gastric pH) — must be confirmed low (acidic) for the diagnosis; a high gastrin from acid-suppressing drugs looks similar but occurs with low acid.
  • Chromogranin A — a general neuroendocrine-tumour marker, often also raised.
  • Calcium and other hormones — screen for the inherited MEN1 syndrome.

Symptoms

  • Severe, multiple or recurrent peptic ulcers
  • Ulcers that persist despite standard treatment, or in unusual places
  • Abdominal pain and heartburn
  • Diarrhoea
  • Nausea and, sometimes, weight loss
  • Ulcer complications such as bleeding (black stools, vomiting blood)

Related symptoms

When to discuss with a doctor

Suspect and investigate Zollinger-Ellison syndrome when peptic ulcers are unusually severe, multiple, recurrent despite proper treatment, in unusual locations, or come with diarrhoea — particularly without the usual causes (H. pylori infection, anti-inflammatory painkillers). Because a high gastrin from common acid-suppressing medicines mimics the picture, confirming the diagnosis needs specialist testing, often after adjusting those medicines under supervision, plus imaging to find the tumour. Ulcer complications such as bleeding are emergencies. The condition is very treatable once recognised. Mediora.AI can surface a markedly raised gastrin and flag the stubborn-ulcer pattern from your results, but confirming Zollinger-Ellison syndrome and locating the tumour is a specialist assessment — the interpretation belongs with your doctor.

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