gastrinoma

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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:

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

Last reviewed 01/2018

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