Extrapulmonary Neuroendocrine Carcinomas: Current Management and Future Perspectives
Neuroendocrine carcinomas (NECs) are poorly differentiated and highly aggressive epithelial neuroendocrine neoplasms. The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, follo...
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| Veröffentlicht in: | Journal of clinical medicine 2023-12, Vol.12 (24), p.7715 |
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| creator | Stumpo, Sara Formelli, Maria Giovanna Persano, Irene Parlagreco, Elena Lauricella, Eleonora Rodriquenz, Maria Grazia Guerrera, Luigi Pio Zurlo, Ina Valeria Campana, Davide Brizzi, Maria Pia Cives, Mauro La Salvia, Anna Lamberti, Giuseppe |
| description | Neuroendocrine carcinomas (NECs) are poorly differentiated and highly aggressive epithelial neuroendocrine neoplasms. The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, followed by the genitourinary (GU) system and gynecological tract. As a result of their rarity, there is scant evidence to guide treatment recommendations, and a multidisciplinary approach is essential for the management of such patients. Platinum-based chemotherapy currently represents the standard of care for EP-NECs of any site, mirroring the management of small-cell lung cancer (SCLC), but further approaches are still under investigation. Indeed, ongoing trials evaluating targeted therapies, immune checkpoint inhibitors (ICIs), and radionuclide therapy could provide potentially breakthrough therapeutic options. Given the relative dearth of evidence-based literature on these orphan diseases, the aim of this review is to provide an overview of the pathology and current treatment options, as well as to shed light on the most pressing unmet needs in the field. |
| doi_str_mv | 10.3390/jcm12247715 |
| format | Article |
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The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, followed by the genitourinary (GU) system and gynecological tract. As a result of their rarity, there is scant evidence to guide treatment recommendations, and a multidisciplinary approach is essential for the management of such patients. Platinum-based chemotherapy currently represents the standard of care for EP-NECs of any site, mirroring the management of small-cell lung cancer (SCLC), but further approaches are still under investigation. Indeed, ongoing trials evaluating targeted therapies, immune checkpoint inhibitors (ICIs), and radionuclide therapy could provide potentially breakthrough therapeutic options. Given the relative dearth of evidence-based literature on these orphan diseases, the aim of this review is to provide an overview of the pathology and current treatment options, as well as to shed light on the most pressing unmet needs in the field.</description><identifier>ISSN: 2077-0383</identifier><identifier>EISSN: 2077-0383</identifier><identifier>DOI: 10.3390/jcm12247715</identifier><identifier>PMID: 38137784</identifier><language>eng</language><publisher>Switzerland: MDPI AG</publisher><subject>Cancer ; Care and treatment ; Chemotherapy ; Clinical medicine ; Clinical trials ; Epidemiology ; Esophagus ; Lung cancer ; Medical prognosis ; Metastasis ; Mutation ; Neuroendocrine tumors ; Peptides ; Protein expression ; Radiation therapy ; Surgery</subject><ispartof>Journal of clinical medicine, 2023-12, Vol.12 (24), p.7715</ispartof><rights>COPYRIGHT 2023 MDPI AG</rights><rights>2023 by the authors. 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/). 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The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, followed by the genitourinary (GU) system and gynecological tract. As a result of their rarity, there is scant evidence to guide treatment recommendations, and a multidisciplinary approach is essential for the management of such patients. Platinum-based chemotherapy currently represents the standard of care for EP-NECs of any site, mirroring the management of small-cell lung cancer (SCLC), but further approaches are still under investigation. Indeed, ongoing trials evaluating targeted therapies, immune checkpoint inhibitors (ICIs), and radionuclide therapy could provide potentially breakthrough therapeutic options. 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| subjects | Cancer Care and treatment Chemotherapy Clinical medicine Clinical trials Epidemiology Esophagus Lung cancer Medical prognosis Metastasis Mutation Neuroendocrine tumors Peptides Protein expression Radiation therapy Surgery |
| title | Extrapulmonary Neuroendocrine Carcinomas: Current Management and Future Perspectives |
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