Neuroendocrine neoplasms: Evolving and future treatments

Abstract

Neuroendocrine neoplasms (NENs) are relatively rare neoplasms. Most NENs are slowly progressing malignancies with prolonged survival even in the presence of advanced metastatic disease, implying an increased need for additional therapies on failure of those initially administered. However, a subset of NENs may display truly aggressive behavior, often refractory to current chemotherapeutic regimens, underlining the need for more appropriate schemes. Following these unmet needs, new therapies are emerging based on advances of NEN pathogenesis, along with modifications of those previously used to improve their efficacy. Long-acting somatostatin analogs also in above-label doses, molecular and peptide receptor radionuclide targeted treatments, chemotherapy, and immunotherapy are available options, but their appropriate sequence is still to be defined, and the choice of monotherapy or combination therapy should be individualized. Advances in the molecular pathogenesis of NENs and larger prospective studies will shed light on biomolecular tumor markers, suggesting the appropriate sequence of treatment in individual patients. © 2021 Elsevier Lt