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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| Format: | Article |
| Language: | English |
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MDPI AG
2022-03-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/5/1461 |
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| author | Elisa Andrini Paola Valeria Marchese Dario De Biase Cristina Mosconi Giambattista Siepe Francesco Panzuto Andrea Ardizzoni Davide Campana Giuseppe Lamberti |
| author_facet | Elisa Andrini Paola Valeria Marchese Dario De Biase Cristina Mosconi Giambattista Siepe Francesco Panzuto Andrea Ardizzoni Davide Campana Giuseppe Lamberti |
| author_sort | Elisa Andrini |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T20:34:07Z |
| format | Article |
| id | doaj.art-8039976617704c298ee729a6121d7a90 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T20:34:07Z |
| publishDate | 2022-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-8039976617704c298ee729a6121d7a902023-11-23T23:16:14ZengMDPI AGJournal of Clinical Medicine2077-03832022-03-01115146110.3390/jcm11051461Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and ChallengesElisa Andrini0Paola Valeria Marchese1Dario De Biase2Cristina Mosconi3Giambattista Siepe4Francesco Panzuto5Andrea Ardizzoni6Davide Campana7Giuseppe Lamberti8Department of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi University Hospital, ENETS Center of Excellence, 40138 Bologna, ItalyDepartment of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi University Hospital, ENETS Center of Excellence, 40138 Bologna, ItalyDepartment of Pharmacy and Biotechnology, Molecular Diagnostic Unit, University of Bologna, Viale Ercolani 4/2, 40138 Bologna, ItalyDepartment of Radiology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, University of Bologna, 40138 Bologna, ItalyRadiation Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40138 Bologna, ItalyDigestive Disease Unit, ENETS Center of Excellence of Rome, Sant’Andrea University Hospital, 00189 Rome, ItalyDepartment of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi University Hospital, ENETS Center of Excellence, 40138 Bologna, ItalyDepartment of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi University Hospital, ENETS Center of Excellence, 40138 Bologna, ItalyDepartment of Experimental, Diagnostic and Specialty Medicine, Sant’Orsola-Malpighi University Hospital, ENETS Center of Excellence, 40138 Bologna, ItalyLarge 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.https://www.mdpi.com/2077-0383/11/5/1461LCNECICIsnext-generation sequencingRB1TP53targeted therapies |
| spellingShingle | Elisa Andrini Paola Valeria Marchese Dario De Biase Cristina Mosconi Giambattista Siepe Francesco Panzuto Andrea Ardizzoni Davide Campana Giuseppe Lamberti Large Cell Neuroendocrine Carcinoma of the Lung: Current Understanding and Challenges Journal of Clinical Medicine LCNEC ICIs next-generation sequencing RB1 TP53 targeted therapies |
| 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 | LCNEC ICIs next-generation sequencing RB1 TP53 targeted therapies |
| url | https://www.mdpi.com/2077-0383/11/5/1461 |
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