Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis
Abstract 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 t...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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BMC
2022-06-01
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| Series: | Orphanet Journal of Rare Diseases |
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| Online Access: | https://doi.org/10.1186/s13023-022-02366-x |
| _version_ | 1811258039915773952 |
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| author | Yeye Chen Jiaqi Zhang Mengxin Zhou Chao Guo Shanqing Li |
| author_facet | Yeye Chen Jiaqi Zhang Mengxin Zhou Chao Guo Shanqing Li |
| author_sort | Yeye Chen |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-04-12T18:07:09Z |
| format | Article |
| id | doaj.art-3c67ce3386de4b369c1ffe0bf9270306 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-12T18:07:09Z |
| publishDate | 2022-06-01 |
| publisher | BMC |
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| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-3c67ce3386de4b369c1ffe0bf92703062022-12-22T03:21:57ZengBMCOrphanet Journal of Rare Diseases1750-11722022-06-011711810.1186/s13023-022-02366-xReal-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysisYeye Chen0Jiaqi Zhang1Mengxin Zhou2Chao Guo3Shanqing Li4Department of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeDepartment of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical CollegeAbstract 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.https://doi.org/10.1186/s13023-022-02366-xThymic neuroendocrine tumorsClinicopathological featuresPrognosis |
| spellingShingle | Yeye Chen Jiaqi Zhang Mengxin Zhou Chao Guo Shanqing Li Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis Orphanet Journal of Rare Diseases Thymic neuroendocrine tumors Clinicopathological features Prognosis |
| 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 | Thymic neuroendocrine tumors Clinicopathological features Prognosis |
| url | https://doi.org/10.1186/s13023-022-02366-x |
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