ABSTRACT
Almost 50% of neuroendocrine tumors are metastatic at diagnosis. Treatment in metastatic neuroendocrine tumors depends on whether the tumor is functional or not and its histological grade (G1-G3). Today, there are somatostatin analogs (octreotide, lanreotide), interferon, chemotherapy, everolimus, sunitinib and other targeted therapy options in metastatic disease. The use of immune checkpoint inhibitors in neuroendocrine neoplasms is limited, as these cancers typically have low tumor mutational burden, low apoptosis ligand-1 expression, and low lymphocyte infiltration.
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