Néoplasies neuro-endocrines broncho-pulmonaires primitives : tumeurs carcinoïdes et carcinomes neuro-endocrines à grandes cellules: Primary pulmonary neuroendocrine neoplasms : carcinoid tumors and large-cell neuro-endocrine carcinomas

Abstract

Primary pulmonary neuroendocrine neoplasms comprise several distinct clinical entities, corresponding to four main subtypes: well differentiated neuroendocrine tumors including typical carcinoid tumors, of low grade malignancy and 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, 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. Peptide receptor radionuclide therapy with 177Lu-DOTATATE in patients with somatostatin receptor-expressing neuroendocrine tumors is an interesting option not yet validated for routine use.In case of advanced, metastatic large-cell neuroendocrine carcinoma, platinum and etoposide combination is generally used in front line. No established second line is available. Results of the IFCT-FFCD-GERCOR “NIPINEC trial” evaluating nivolumab, with or without ipilimumab in the second- and third-line setting are awaiting. Molecular analysis of a panel of oncogenic alterations could guide therapeutic decision.© 2021 SPLF. Published by Elsevier Masson SAS. All rights reserved.