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Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges

Large cell neuroendocrine carcinoma of the lung (LCNEC) is a rare and highly aggressive type of lung cancer, with a complex biology that shares similarities with both small-cell lung cancer (SCLC) and non-small-cell lung cancer (NSCLC). The prognosis of LCNEC is poor, with a median overall survival...

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Autores principales: Andrini, Elisa, Marchese, Paola Valeria, De Biase, Dario, Mosconi, Cristina, Siepe, Giambattista, Panzuto, Francesco, Ardizzoni, Andrea, Campana, Davide, Lamberti, Giuseppe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8911276/
https://www.ncbi.nlm.nih.gov/pubmed/35268551
http://dx.doi.org/10.3390/jcm11051461
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author Andrini, Elisa
Marchese, Paola Valeria
De Biase, Dario
Mosconi, Cristina
Siepe, Giambattista
Panzuto, Francesco
Ardizzoni, Andrea
Campana, Davide
Lamberti, Giuseppe
author_facet Andrini, Elisa
Marchese, Paola Valeria
De Biase, Dario
Mosconi, Cristina
Siepe, Giambattista
Panzuto, Francesco
Ardizzoni, Andrea
Campana, Davide
Lamberti, Giuseppe
author_sort Andrini, Elisa
collection PubMed
description Large cell neuroendocrine carcinoma of the lung (LCNEC) is a rare and highly aggressive type of lung cancer, with a complex biology that shares similarities with both small-cell lung cancer (SCLC) and non-small-cell lung cancer (NSCLC). The prognosis of LCNEC is poor, with a median overall survival of 8–12 months. The diagnosis of LCNEC requires the identification of neuroendocrine morphology and the expression of at least one of the neuroendocrine markers (chromogranin A, synaptophysin or CD56). In the last few years, the introduction of next-generation sequencing allowed the identification of molecular subtypes of LCNEC, with prognostic and potential therapeutic implications: one subtype is similar to SCLC (SCLC-like), while the other is similar to NSCLC (NSCLC-like). Because of LCNEC rarity, most evidence comes from small retrospective studies and treatment strategies that are extrapolated from those adopted in patients with SCLC and NSCLC. Nevertheless, limited but promising data about targeted therapies and immune checkpoint inhibitors in patients with LCNEC are emerging. LCNEC clinical management is still controversial and standardized treatment strategies are currently lacking. The aim of this manuscript is to review clinical and molecular data about LCNEC to better understand the optimal management and the potential prognostic and therapeutic implications of molecular subtypes.
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spelling pubmed-89112762022-03-11 Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges Andrini, Elisa Marchese, Paola Valeria De Biase, Dario Mosconi, Cristina Siepe, Giambattista Panzuto, Francesco Ardizzoni, Andrea Campana, Davide Lamberti, Giuseppe J Clin Med Review Large cell neuroendocrine carcinoma of the lung (LCNEC) is a rare and highly aggressive type of lung cancer, with a complex biology that shares similarities with both small-cell lung cancer (SCLC) and non-small-cell lung cancer (NSCLC). The prognosis of LCNEC is poor, with a median overall survival of 8–12 months. The diagnosis of LCNEC requires the identification of neuroendocrine morphology and the expression of at least one of the neuroendocrine markers (chromogranin A, synaptophysin or CD56). In the last few years, the introduction of next-generation sequencing allowed the identification of molecular subtypes of LCNEC, with prognostic and potential therapeutic implications: one subtype is similar to SCLC (SCLC-like), while the other is similar to NSCLC (NSCLC-like). Because of LCNEC rarity, most evidence comes from small retrospective studies and treatment strategies that are extrapolated from those adopted in patients with SCLC and NSCLC. Nevertheless, limited but promising data about targeted therapies and immune checkpoint inhibitors in patients with LCNEC are emerging. LCNEC clinical management is still controversial and standardized treatment strategies are currently lacking. The aim of this manuscript is to review clinical and molecular data about LCNEC to better understand the optimal management and the potential prognostic and therapeutic implications of molecular subtypes. MDPI 2022-03-07 /pmc/articles/PMC8911276/ /pubmed/35268551 http://dx.doi.org/10.3390/jcm11051461 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Andrini, Elisa
Marchese, Paola Valeria
De Biase, Dario
Mosconi, Cristina
Siepe, Giambattista
Panzuto, Francesco
Ardizzoni, Andrea
Campana, Davide
Lamberti, Giuseppe
Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title_full Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title_fullStr Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title_full_unstemmed Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title_short Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges
title_sort large cell neuroendocrine carcinoma of the lung: current understanding and challenges
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8911276/
https://www.ncbi.nlm.nih.gov/pubmed/35268551
http://dx.doi.org/10.3390/jcm11051461
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