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Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis
BACKGROUND: Thymic neuroendocrine tumors comprise a heterogeneous group of rare diseases. This study aimed to investigate the real-world clinicopathological features and treatment outcomes of thymic neuroendocrine tumors. RESULTS: A total of 104 patients diagnosed with thymic neuroendocrine tumors i...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9169285/ https://www.ncbi.nlm.nih.gov/pubmed/35668481 http://dx.doi.org/10.1186/s13023-022-02366-x |
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author | Chen, Yeye Zhang, Jiaqi Zhou, Mengxin Guo, Chao Li, Shanqing |
author_facet | Chen, Yeye Zhang, Jiaqi Zhou, Mengxin Guo, Chao Li, Shanqing |
author_sort | Chen, Yeye |
collection | PubMed |
description | BACKGROUND: Thymic neuroendocrine tumors comprise a heterogeneous group of rare diseases. This study aimed to investigate the real-world clinicopathological features and treatment outcomes of thymic neuroendocrine tumors. RESULTS: A total of 104 patients diagnosed with thymic neuroendocrine tumors in a single institution from 1983 to 2021 were eligible. Fourteen (13.46%) and 28 (26.92%) patients diagnosed with thymic neuroendocrine tumors suffered from multiple endocrine neoplasia and ectopic adrenocorticotropic hormone syndrome, respectively. Ninety-seven (93.27%) patients underwent surgical resection, including 79 (81.44%) with radical resection. Except for 5 patients lost during follow-up, the 1-, 3- and 5-year overall survival rates were 91.8%, 70.2% and 54.6%, respectively. The median overall survival was 61.57 months. Multivariate analysis revealed that years at diagnosis (HR 0.559, 95% CI 0.364–0.857, p = 0.008), radical resection (HR 2.860, 95% CI 1.392–5.878, p = 0.004), pathological grade (HR 1.963, 95% CI 1.058–3.644, p = 0.033) and Masaoka–Koga stage (HR 2.250, 95% CI 1.548–3.272, p = 0.000) exerted significant differences in overall survival among 99 patients. In the surgery group, multivariate Cox regression analysis exhibited significant overall survival differences in years at diagnosis (HR 0.563, 95% CI 0.367–0.866, p = 0.009), neoadjuvant therapy (HR 0.248, 95% CI 0.071–0.872, p = 0.030), radical resection (HR 3.674, 95% CI 1.685–8.008, p = 0.001), pathological grade (HR 2.082, 95% CI 1.098–3.947, p = 0.025) and Masaoka–Koga stage (HR 2.445, 95% CI 1.607–3.719, p = 0.000). CONCLUSIONS: Radical resection and Masaoka–Koga stage were independent prognostic factors for the survival of patients with thymic neuroendocrine tumors. Systemic therapy and integrated management of patients with advanced-stage disease require high-level clinical evidence. |
format | Online Article Text |
id | pubmed-9169285 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-91692852022-06-07 Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis Chen, Yeye Zhang, Jiaqi Zhou, Mengxin Guo, Chao Li, Shanqing Orphanet J Rare Dis Research BACKGROUND: Thymic neuroendocrine tumors comprise a heterogeneous group of rare diseases. This study aimed to investigate the real-world clinicopathological features and treatment outcomes of thymic neuroendocrine tumors. RESULTS: A total of 104 patients diagnosed with thymic neuroendocrine tumors in a single institution from 1983 to 2021 were eligible. Fourteen (13.46%) and 28 (26.92%) patients diagnosed with thymic neuroendocrine tumors suffered from multiple endocrine neoplasia and ectopic adrenocorticotropic hormone syndrome, respectively. Ninety-seven (93.27%) patients underwent surgical resection, including 79 (81.44%) with radical resection. Except for 5 patients lost during follow-up, the 1-, 3- and 5-year overall survival rates were 91.8%, 70.2% and 54.6%, respectively. The median overall survival was 61.57 months. Multivariate analysis revealed that years at diagnosis (HR 0.559, 95% CI 0.364–0.857, p = 0.008), radical resection (HR 2.860, 95% CI 1.392–5.878, p = 0.004), pathological grade (HR 1.963, 95% CI 1.058–3.644, p = 0.033) and Masaoka–Koga stage (HR 2.250, 95% CI 1.548–3.272, p = 0.000) exerted significant differences in overall survival among 99 patients. In the surgery group, multivariate Cox regression analysis exhibited significant overall survival differences in years at diagnosis (HR 0.563, 95% CI 0.367–0.866, p = 0.009), neoadjuvant therapy (HR 0.248, 95% CI 0.071–0.872, p = 0.030), radical resection (HR 3.674, 95% CI 1.685–8.008, p = 0.001), pathological grade (HR 2.082, 95% CI 1.098–3.947, p = 0.025) and Masaoka–Koga stage (HR 2.445, 95% CI 1.607–3.719, p = 0.000). CONCLUSIONS: Radical resection and Masaoka–Koga stage were independent prognostic factors for the survival of patients with thymic neuroendocrine tumors. Systemic therapy and integrated management of patients with advanced-stage disease require high-level clinical evidence. BioMed Central 2022-06-06 /pmc/articles/PMC9169285/ /pubmed/35668481 http://dx.doi.org/10.1186/s13023-022-02366-x Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Chen, Yeye Zhang, Jiaqi Zhou, Mengxin Guo, Chao Li, Shanqing Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title | Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title_full | Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title_fullStr | Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title_full_unstemmed | Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title_short | Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
title_sort | real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9169285/ https://www.ncbi.nlm.nih.gov/pubmed/35668481 http://dx.doi.org/10.1186/s13023-022-02366-x |
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