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Zollinger-Ellison syndrome

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The Zollinger-Ellison syndrome describes the association of:

  • gastrin-producing tumours
  • gastric hypersecretion
  • severe peptic ulcer disease

Usually, the tumour is situated in the pancreas but may arise in the upper small intestine or gastric antrum. 60% of the tumours are malignant. 10% are multiple

Patients with the Zollinger-Ellison syndrome may develop diarrhoea and malabsorption due to inhibition of pancreatic lipase by the excessive gastric acid. This syndrome occurs in about 0.1% of patients with duodenal ulcer disease.

Gastrinomas may occurs as part of a multiple endocrine neoplasia syndrome and so screening for pituitary adenomas, parathyroid tumours and other pancreatic tumours may be indicated

  • approximately 20–25% of gastrinoma patients have MEN-I syndrome (1)

Diagnosis and investigation of gatrinomas:

  • high fasting plasma gastrin
  • high gastric acid secretion
  • diminished response to pentagastrin
  • demonstrable pancreatic or gastrointestinal tumour - by CT or venous sampling for gastrin
  • more than 90% of gastrinomas have somatostatin receptors, and somatostatin receptor scintigraphy has been reported to be a especially sensitive method to image gastrinomas

Management:

  • management is directed by a specialist
  • most patients are managed with proton pump inhibitors
  • octreotide can also control acid hypersecretion in patients with Zollinger-Ellison syndrome - this treatment modality has a favourable outcome on the patients' prognosis and survival (1)
  • total gastrectomy and parietal cell vagotomy are only seldom necessary
  • surgical resection of the primary tumour(s) may improve prognosis and even result in a complete cure (1)
    • note also that there is a general consensus that curative surgery should also be aimed for in metastatic disease, including metastatic disease ‘localized’ to the liver (1)
  • systemic chemotherapy is indicated in patients with metastatic disease
  • liver transplantation may be considered in patients with no extrahepatic metastases

Notes:

  • in diagnosing gastrinoma, the measurement of basal and maximal gastric acid output is mandatory to distinguish it from secondary hypergastrinaemia
  • at the time of diagnosis of a gastrinoma, 60–80% have metastasized to regional lymph nodes or to the liver.

Reference:

  1. de Herder WW and Lamberts SWJ. Best Practice & Research Clinical Endocrinology & Metabolism 2004; Volume 18(4): 477-495.

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