Advances in Targeted Therapy for Patients with Neuroendocrine Tumours

Abstract

Neuroendocrine neoplasms are heterogenous tumours with diverse biological behaviour. Well-differentiated neuroendocrine tumours comprise the vast majority of these malignancies. Though a subset of patients may possess indolent disease, which can be observed, most patients require systemic therapy at some point. The treatment armamentarium for patients with metastatic or advanced well-differentiated neuroendocrine tumours has expanded significantly over recent years, with multiple regulatory approvals for systemic therapies. Though peptide receptor radionuclide therapy has been a major addition to this armamentarium, several targeted therapies have also been successfully developed. Herein, we discuss the approved targeted therapies sunitinib and everolimus and highlight the clinical experience with targeted therapies in development. We focus largely on novel receptor tyrosine kinases targeting vascular endothelial growth factor, inhibitors of cell-cycle drivers, metabolic-pathway inhibitors and chemotherapy, and immune-modulating agents targeting the somatostatin receptor.