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 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.

Case report: Radiofrequency ablation combined with biopsy for Cushing’s syndrome due to ectopic ACTH lesions in the lung

“…Compared with RFA of other parenchymal organs, the gas around the lung lesion can restrict the heat conduction during the ablation process, and the energy can be more easily gathered inside the lesion, which makes the ablation more efficient and effective; (5) When a biopsy is required, the biopsy needle and RF probe can be placed at the same time to avoid secondary puncture injury, and the small blood vessels damaged by the puncture cut can be closed with RF ablation heating to reduce the risk of bleeding. (6) Radiofrequency ablation as a local minimally invasive ablation treatment, combined with other therapeutic methods sometimes can improve the efficacy, such as radiotherapy, holistic therapy (depending on genetic test results, choose to use chemotherapy or molecularly targeted drug therapy) and so on.…”

“…In patients who are clinically diagnosed with Cushing's syndrome in combination with laboratory tests, hormone experiments and the evident ectopic ACTH lesions after imaging, conventional treatments such as drug therapy and surgical resection are challenging to implement (5,6). Some techniques can be used to target the lesions and control systemic symptoms,such as radiotherapy, chemotherapy, and radionuclides (5).…”

Thymic neuroendocrine neoplasms—what we know, what we don’t know, and what to do about it

“…Thymic (5). And in June 2022, Chen et al reported their retrospective analysis of a remarkable 104 cases in a single center (6).…”

“…We would like to add two surgical papers to the discussion: Wang et al in 2016 published 18 t-NEN cases in this journal ( 5 ). And in June 2022, Chen et al reported their retrospective analysis of a remarkable 104 cases in a single center ( 6 ).…”

Significance of the Surgical Treatment with Lymph Node Dissection for Neuroendocrine Tumors of Thymus

“…Tumor size [ 7 ] and histopathologic subtype [ 8 ] are reported as prognostic factors. Although lymph nodes and distant metastases appear with high probability, the efficacy of chemotherapy or radiotherapy is reported to be insufficient for NETTs, and complete resection is important [ 3 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 ]. The prognosis has become poor in the progressive stage, but this can be explained by the confounding effect of the possibility of complete resection [ 10 ].…”