Abstract

To describe recent advances in the treatment of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). A review of the published English language literature on GEP-NET therapy with a focus on practice-changing clinical trials. Somatostatin analog (SSA) treatment remains a cornerstone of GEP-NET therapy, primarily for patients with hormonally functional tumors and midgut carcinoids. The biologic agents everolimus and sunitinib have similar tumor-stabilizing effects in pancreatic NETs and are both approved to treat progressive low-intermediate-grade tumors. Their role in nonpancreatic NETs remains controversial. Cytotoxic chemotherapy is effective against pancreatic NETs, but modern prospective data is lacking. Radiolabeled SSAs will likely become more widely available once phase III randomized studies are completed. New treatment options for GEP-NETs have become available and highlight the necessity of developing predictive biomarkers that will allow for appropriate and individualized therapy selection.

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