Neuroendocrine Neoplasia Management | 2021
New Approaches in Medical Therapies
Abstract
Neuroendocrine tumors (NETs) represent a group of rare neoplasms that can arise throughout the body from cells of the diffuse endocrine system. The only curative treatment is surgery; however, most patients are diagnosed with metastatic disease, therefore, preclinical and clinical research have investigated the molecular pathways associated with NETs development, which lead to discovering multiple long-term systemic therapeutic options. Evidence-based treatment options include somatostatin analogs, everolimus (mTOR inhibitor), sunitinib (tyrosine kinase inhibitor), peptide receptor radionuclide therapy, and locoregional therapies. However, different drugs are currently under investigation, of which some molecules are already used in NETs, while others are approved in other tumors. New treatment options under investigation are new somatostatin analog (pasireotide) or new tyrosine kinase inhibitors (pazopanib, cabozantinib, lenvatinib, axitinib, surufatinib). Moreover, immune checkpoint inhibitors have also been investigated in NETs, with some encouraging results in well-differentiated tumors, especially for lung NETs. Clinical studies have also investigated the efficacy of epigenetic drugs (panobinostat and LSD-1 inhibitor) and cyclin-dependent 4/6 inhibitors (abemaciclib and palbociclib) in small population, but more evidence are needed to show the efficacy of these categories of treatments in NETs.