Orphanet journal of rare diseases · 2022
Real-world clinicopathological features and outcome of thymic neuroendocrine tumors: a retrospective single-institution analysis.
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Chen Yeye, Zhang Jiaqi, Zhou Mengxin, Guo Chao, Li Shanqing
Аннотация
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.
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.