Clinical Advances in Hematology & OncologyTreatment Landscape of Advanced High-Grade Neuroendocrine Neoplasms

The prognosis and therapy of grade 3 neuroendocrine neoplasms (NEN G3) depend on subgroup traits such as differentiation, proliferation rate, molecular profile, uptake on somatostatin receptor imaging, and primary site. Clinicians who treat this disease now have a better grasp of the prognosis and course of treatment for each entity thanks to the 2017 World Health Organization guidelines that split NEN G3 into neuroendocrine carcinomas (NEC) and NET G3. The distinction between NET G3 and NEC in upcoming trials and cancer registries will further improve prognosis and therapy options. Referral to a tertiary center is still of the utmost necessity at this time, considering the complexities of this disease. For the reasons described in this article, a referral also enables expert pathologic review, which is crucial. Further trials are undoubtedly necessary, considering the molecular heterogeneity of NEN G3.