Gastrinomas may be benign sporadic, malignant metastatic, and part of MEN-I. They occur in the pancreas, duodenum, jejunum, lymph nodes, mesentery, gallbladder, and ovary. Patients with peptic ulcer disease should have a screening gastrin and if elevated (> 150 pg/ml) undergo a secretin stimulation test (2ug/kg with a rise in gastrin of > 100 pg/ml) to distinguish hypergastrinemia from other causes such as atrophic gastritis, G cell hyperplasia, hypercalcemia, hyperparathyroidism, pernicious anemia and MEN-1. Diagnosis is certain if the gastric acid output is > 15mmol/h, ph is <3. CT, MRI and somatostatin receptor scintigraphy yield the best results for localization. Small tumors require portal venous sampling enhanced by secretin or calcium stimulation for localization. 68Ga-DOTATATE PET, detects 75-85%, 68Ga-DOTANOC PET has a sensitivity of 93.5%. Treatment goals are control of acid hypersecretion with PPIs, resection of the tumor and managing the metastatic disease. MEN-1 requires identification of the tumor producing the gastrin and attention to the pituitary and parathyroid tumors which contribute to the mortality. For malignant metastatic disease the survival can be increased with liver directed therapies, debulking surgery, use of radiofrequency ablation and chemo and bland embolization. Progression free survival (PFS) is increased using somatostatin analog therapy. Peptide receptor radioligand therapy (PRRT) prolongs PFS and enhances quality of life. Second line therapy includes MTOR and Tyrosine kinase inhibitors. Chemotherapy achieves responses in >50%.
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Diagnosis and Treatment of Gastrinoma in the Era of Proton Pump InhibitorsM Banasch et al. Wien Klin Wochenschr 119 (19-20), 573-8. PMID 17985090.The Zollinger-Ellison syndrome is characterized pathophysiologically by a significant hypergastrinemia derived from a gastrin-secreting neuroendocrine tumor with a primar …
Pancreatic Polypeptide (PPoma)A Vinik et al. PMID 25905295. - ReviewPancreatic polypeptide (PP) is a 36 amino acid peptide 93% of which is produced by F cells in the pancreas. PP has no real known actions (it stimulates pancreatic enzyme …
A Primary Hepatic Gastrinoma Accompanied by Hyperplasia of Multi-Nodular Brunner's GlandsT Hagi et al. Surg Case Rep 3 (1), 118. PMID 29181825.We report a case of primary hepatic gastrinoma in a patient who has been cured for 4 years postoperatively. The diagnosis was somewhat difficult due to the coexisting, mu …
Zollinger-Ellison SyndromePD Hung et al. Curr Treat Options Gastroenterol 6 (2), 163-170. PMID 12628075.Zollinger-Ellison syndrome (ZES) is caused by a gastrin-producing tumor called a gastrinoma, which results in gastric acid hypersecretion. Gastrin stimulates the parietal …
Novel Association of Duodenal Gastrinoma and Atrophic Gastritis: Case Report and Literature ReviewI Lendel et al. Endocr Pract 13 (7), 770-5. PMID 18194935. - ReviewPatients with atrophic gastritis and associated hypergastrinemia are known to have a high frequency of hypergastrinemia-induced gastric carcinoid tumors, some of which ar …