Immunotherapy with checkpoint inhibitors targeting the programmed cell death protein 1 (PD-1)/programmed death-ligand 1 (PD-L1) axis or Cytotoxic T-lymphocyte-associated protein 4 (CTLA-4) has shown only modest activity in neuroendocrine tumors (NET) and neuroendocrine carcinomas (NEC). This evidence concerns the gene CTLA4 and neuroendocrine carcinoma.