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

Abstract

Primary pulmonary neuroendocrine neoplasms comprise several distinct clinical entities, corresponding to four main histological subtypes: well differentiated neuroendocrine tumors including typical carcinoid tumors, of low grade malignancy and atypical carcinoids of intermediate grade malignancy; and neuro-endocrine carcinomas with either small or large cells, of high grade malignancy.The pre-treatment workup of carcinoid tumors relies 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. However, for tumors that express RB or harbor KRAS or STK11 mutations, platinum and gemcitabine or platinum and taxane combination may be used. No established second line is available, but treatments used in NSCLC can be suggested, except pemetrexed.1877-1203/© 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.