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Zollinger–Ellison syndrome is a rare clinical syndrome caused by gastrin-secreting tumors (gastrinomas), leading to gastric acid hypersecretion, multiple/refractory peptic ulcers, and secretory diarrhea.

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https://www.youtube.com/watch?v=Nl_Qgqe-jTQ

Etiopathogenesis

• Gastrinoma = neuroendocrine tumor of G-cells secreting gastrin.
• Location (“gastrinoma triangle”):
  • Junction of cystic duct and common bile duct.
  • Second and third parts of duodenum.
  • Pancreatic head.
• 80–90% tumors are in this triangle.
• Sporadic (60–70%) or associated with MEN 1 syndrome (20–25%).
• Malignancy: ~60% are malignant with liver metastasis.

Pathophysiology

• ↑ Gastrin → ↑ parietal cell mass and gastric acid → multiple, recurrent, atypical ulcers.
• Ulcers may occur in unusual locations (distal duodenum, jejunum).
• High acid load inactivates pancreatic enzymes → fat malabsorption → diarrhea/steatorrhea.

Clinical Features

• Severe, recurrent or multiple peptic ulcers (often distal to duodenal bulb).
• Ulcers refractory to standard therapy.
• Chronic diarrhea and steatorrhea.
• Abdominal pain, GI bleeding.