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Gastrinoma

Last updated: October 12, 2020

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A gastrinoma (Zollinger-Ellison syndrome) is a gastrin-secreting neuroendocrine tumor that is most often localized to the duodenum and pancreas. Most gastrinomas occur sporadically, but some are associated with other endocrine neoplasias (e.g., pituitary adenomas, parathyroid adenomas, insulinomas). Although some gastrinomas are benign, more than half of all gastrinomas are malignant. Gastrinomas release high levels of gastrin, which then increases the production of gastric acid. Patients typically present with recurrent, therapy-resistant peptic ulcer disease and diarrhea. Patients with gastrinomas have low gastric pH and elevated serum gastrin. Furthermore, serum gastrin levels increase with the administration of secretin (positive secretin stimulation test). Surgical resection of the tumor is indicated in patients with localized disease. Proton pump inhibitors (PPIs) and octreotide may be used to control acid secretion.

  • Sex: > (2:1)
  • Age of onset: 30–50 years

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

References:[3][4][5]

References:[1][5][7]

Most patients manifest with recurrent, therapy-resistant peptic ulcer disease.

References:[1][7]

The presence of multiple, large (> 2 cm) ulcers in atypical locations (e.g., the jejunum) should raise suspicion of gastrinoma!

Low gastric pH along with gastrin levels > 1000 pg/mL is virtually sufficient to diagnose gastrinoma. If gastrin levels are less than 1000 pg/mL, a secretin stimulation test must be performed!

References:[4][5][7]

In approximately 50% of cases, the tumor has already metastasized at the time of diagnosis!References:[3][10]

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