Molecular Pathology of Neuroendocrine Tumor: The Era of Targeted Therapeutics

Neuroendocrine neoplasms are heterogeneous neoplasms derived from diverse organs and tissues. Currently systemic treatments include cytotoxic agents, anti-somatostatin compounds, immunotherapy, and targeted agents. The selection of therapy is based on the grade of the tumor with high-grade neoplasms receiving cytotoxic therapies and lower-grade ones being treated with bioactive compounds including somatostatin analogues and targeted therapies against mTOR, angiogenesis, and other molecular targets. Finally, immunotherapy using immune checkpoint inhibitors is an emerging therapeutic option.