Systemic treatment for neuroendocrine non-small cell lung carcinoma: A cases series and a systematic review of the literature

Introduction: Neuroendocrine lung cancer constitutes a continuum from carcinoid tumours (CT) to large cell neuroendocrine (LCNEC) and small-cell carcinomas (SCLC). Except for SCLC, there is no consensual agreement on systemic therapy. The aim of this study is to review our clinical experience among patients with CT and LCNEC in the light of a systematic review of the literature.Methods: A retrospective study of all patients with CT and LCNEC receiving a systemic therapy at Institut Jules Bordet and Erasme Hospital between 01/01/2000-31/12/2020. A systematic review of the literature was per-formed in Ovid Medline.Results: 53 patients (21 CT and 32 LCNEC) were included. Despite limited response rates, patients with CT receiving a carcinoid-like 1st-line regimen (somatostatin analogues (SSA), everolimus, peptide receptor radionuclide therapy (PRRT)) had a numerically longer survival compared to those receiving other type of regimens (median 51.4 vs 18.6 months, respectively; p = 0.17). We observed a similar survival between 1st line SCLC-like vs non-small cell lung cancer (NSCLC)-like schemes in LCNEC (median 11.2 vs 12.6 months, respectively; p = 0.46). The systematic review identified 23 studies (12 prospective, 15 and 8 for CT and LCNEC respectively). For CT, everolimus and SSA led to prolonged disease control with an acceptable toxicity profile, while higher response rates but lower tolerance were associated with PRRT and chemotherapy regimens including oxaliplatine and dacarbazine. For LCNEC, no difference emerged when comparing SCLC-like and NSCLC-like regimens considering response rate, progression-free or overall survival. Conclusions: SSA, everolimus and PRRT present a good therapeutic index for CT, while the role of chemotherapy remains limited to aggressive and rapidly evolving CT. The best type of chemotherapy regimen remains an open question in LCNEC.

Automated summary

This study reviewed the clinical experience of patients with neuroendocrine lung cancer, including carcinoid tumors, large cell neuroendocrine carcinomas, and small-cell carcinomas. The study found that patients with carcinoid tumors who received a carcinoid-like first-line regimen had longer survival compared to those receiving other types of regimens. For large cell neuroendocrine carcinomas, there was no significant difference in survival between SCLC-like and non-small cell lung cancer-like regimens. The systematic review identified different therapeutic options for both types of neuroendocrine lung cancer.