Introduction: Thymic epithelial tumors (TETs) are associated with paraneoplastic/autoimmune (PN/AI) syndromes. Myasthenia gravis is the most common PN/AI syndrome associated with TETs. Methods: The International Thymic Malignancy Interest Group retrospective database was examined to determine (1) baseline and treatment characteristics associated with PN/AI syndromes and (2) the prognostic role of PN/AI syndromes for patients with TETs. The competing risks model was used to estimate cumulative incidence of recurrence (CIR) and the Kaplan‐Meier method was used to calculate overall survival (OS). A Cox proportional hazards model was used for multivariate analysis. Results: A total of 6670 patients with known PN/AI syndrome status from 1951 to 2012 were identified. PN/AI syndromes were associated with younger age, female sex, thymoma histologic type, earlier stage, and an increased rate of total thymectomy and complete resection status. There was a statistically significant lower CIR in the group with a PN/AI syndrome than in the group without a PN/AI syndrome (10‐year CIR 17.3% versus 21.2%, respectively [p = 0.0003]). The OS was improved in the group with a PN/AI syndrome compared to the group without a PN/AI syndrome (median OS 21.6 years versus 17.0 years, respectively [hazard ratio = 0.63, 95% confidence interval: 0.54–0.74, p < 0.0001]). However, in the multivariate model for recurrence‐free survival and OS, PN/AI syndrome was not an independent prognostic factor. Discussion: Previously, there have been mixed data regarding the prognostic role of PN/AI syndromes for patients with TETs. Here, using the largest data set in the world for TETs, PN/AI syndromes were associated with favorable features (i.e., earlier stage and complete resection status) but were not an independent prognostic factor for patients with TETs.

中文翻译：

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副肿瘤综合征和胸腺恶性肿瘤：国际胸腺恶性肿瘤兴趣小组回顾性数据库的检查

简介：胸腺上皮肿瘤 (TET) 与副肿瘤/自身免疫 (PN/AI) 综合征相关。重症肌无力是与 TET 相关的最常见的 PN/AI 综合征。方法：检查国际胸腺恶性肿瘤兴趣小组回顾性数据库以确定（1）与 PN/AI 综合征相关的基线和治疗特征，以及（2）PN/AI 综合征对 TET 患者的预后作用。使用竞争风险模型来估计累积复发率（CIR），并使用Kaplan-Meier法来计算总生存期（OS）。Cox比例风险模型用于多变量分析。结果：从 1951 年到 2012 年，总共确定了 6670 名已知 PN/AI 综合征状态的患者。PN/AI 综合征与年龄较小、女性、胸腺瘤组织学类型、早期阶段以及胸腺全切除率和完全切除状态增加相关。患有 PN/AI 综合征的组的 CIR 显着低于不患有 PN/AI 综合征的组（10 年 CIR 分别为 17.3% 和 21.2% [p = 0.0003]）。与没有 PN/AI 综合征的组相比，有 PN/AI 综合征的组的 OS 有所改善（中位 OS 分别为 21.6 年和 17.0 年 [风险比 = 0.63，95% 置信区间：0.54–0.74，p < 0.0001]）。然而，在无复发生存率和 OS 的多变量模型中，PN/AI 综合征并不是独立的预后因素。讨论：之前，关于 PN/AI 综合征对 TET 患者的预后作用的数据不一。在这里，使用世界上最大的 TET 数据集，PN/AI 综合征与有利特征（即早期阶段和完全切除状态）相关，但并不是 TET 患者的独立预后因素。