Abstract
Pancreatic neuroendocrine neoplasms (PanNENs) are a diverse group of cancers with varying clinical presentations and prognoses due to differences in morphology and clinical stage. Most are non-functional tumors that express somatostatin receptors (SSTRs). Several treatment options have been established for patients with locally advanced or metastatic PanNETs, but the optimal choice of treatment approach and the sequence of available therapies are not yet clearly defined and are currently being studied in multiple ongoing clinical trials. Additionally, new drugs are being researched for PanNET treatment, including immune checkpoint inhibitors, next-generation peptide receptor radionuclide therapy, and other targeted biological therapies. To improve treatment outcomes for patients with PanNETs, a multidisciplinary team should evaluate systemic treatment options. The aim of this article is to review currently available therapies and discuss new and emerging systemic treatment strategies for patients with advanced PanNETs.