Background/Objectives: Thymic neuroendocrine neoplasms (t-NENs) are rare, biologically aggressive malignancies of the anterior mediastinum. Due to their low incidence, clinical evidence remains limited, and treatment recommendations are primarily based on expert opinion and extrapolation from other neuroendocrine tumors. This study aimed to analyze the clinicopathological features, treatment patterns, and survival outcomes of patients with t-NENs treated at a single comprehensive cancer center over 25 years.Methods: A retrospective review was performed on 19 adult patients diagnosed with t-NENs between 2000 and 2024. Data on demographics, histology, treatment intent, modalities used, and outcomes were collected. Survival analyses—of overall survival (OS), disease-free survival (DFS) and progression-free survival (PFS)—were conducted using the Kaplan–Meier method.Results: The median age was 52 years; 63% of patients were male. Atypical carcinoid was the most common histologic subtype (52.6%), followed by large cell neuroendocrine carcinoma (31.6%). Paraneoplastic syndromes, including Cushing’s syndrome, were observed in 26.3% of cases. Radical surgery was performed for 8 patients, but R0 resection was achieved in only 25% of them. Postoperative radiotherapy and chemotherapy were used for 36.8% and 15.8% of patients, respectively. Disease recurrence occurred in 44.4% of curatively treated patients. The median OS for the entire cohort was 127 months; patients treated with curative intent had a significantly longer OS (170 months) compared to those after palliative treatment (33 months). Median PFS in the palliative group was 11 months.Conclusions: t-NENs are aggressive tumors with high risk of recurrence and limited systemic treatment efficacy. Complete surgical resection remains the cornerstone of curative therapy. However, the overall prognosis remains poor, emphasizing the need for novel therapeutic strategies and prospective multicenter studies.
背景/目的：胸腺神经内分泌肿瘤（t-NENs）是前纵隔罕见的、具有侵袭性生物学行为的恶性肿瘤。由于其发病率低，临床证据仍然有限，治疗建议主要基于专家意见和其他神经内分泌肿瘤的推断。本研究旨在分析一家综合性癌症中心25年间收治的t-NENs患者的临床病理特征、治疗模式和生存结局。 方法：对2000年至2024年间诊断为t-NENs的19例成年患者进行回顾性分析。收集了人口统计学、组织学、治疗目的、采用的治疗方式和结局数据。使用Kaplan-Meier法进行总生存期（OS）、无病生存期（DFS）和无进展生存期（PFS）的生存分析。 结果：中位年龄为52岁；63%的患者为男性。不典型类癌是最常见的组织学亚型（52.6%），其次是大细胞神经内分泌癌（31.6%）。26.3%的病例观察到副肿瘤综合征，包括库欣综合征。8例患者接受了根治性手术，但其中仅25%实现了R0切除。术后放疗和化疗的使用率分别为36.8%和15.8%。接受根治性治疗的患者中，44.4%出现疾病复发。整个队列的中位OS为127个月；与接受姑息治疗的患者（33个月）相比，以治愈为目的进行治疗的患者OS显著更长（170个月）。姑息治疗组的中位PFS为11个月。 结论：t-NENs是具有高复发风险和有限全身治疗效果的侵袭性肿瘤。完全手术切除仍然是治愈性治疗的基石。然而，总体预后仍然较差，这强调了需要新的治疗策略和前瞻性多中心研究。
肿瘤(癌症)患者之家