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Review

Gastrinoma

In: Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000–.
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Review

Gastrinoma

Eric Feliberti et al.

Excerpt

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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