Tumeurs neuro-endocrines broncho-pulmonaires primitives: tumeurs carcinoïdes et carcinomes neuro-endocrines à grandes cellules

Abstract

Primary pulmonary neuroendocrine tumors comprise several distinct clinical entities, corresponding to four main subtypes: typical carcinoid tumors, of low grade malignancy; atypical carcinoids of intermediate grade; neuro-endocrine carcinomas with either small or large cells, which are high grade, frequent malignancy.The pre-treatment workup of carcinoid tumors is based on specific recommendations. Any clinical suspicion of an associated functional syndrome must be confirmed by the appropriate blood dosages.In case of metastatic carcinoid tumor, management relies on the control of a possible secretory syndrome by somatostatin analogues, in addition to the oncological treatment, based on the evaluation of tumor progression, with several opportunities: close follow-up, local treatment of metastases, treatment with somatostatin analogues, everolimus, or chemotherapy for tumor with high aggressiveness.In case of advanced, metastatic large-cell neuroendocrine carcinoma, molecular analysis of a panel of oncogenic alterations could guide the chemotherapy strategy; platinum and etoposide combination has historically been used. In the second line, in the absence of any therapeutic standard, the IFCT-FFCD-GERCOR “NIPINEC trial” is recruiting in France in and aims at evaluating nivolumab, with or without ipilimumab in the second- and third-line setting.