Introduction: Thymic epithelial tumors (TETs) are rare malignancies that may be aggressive and difficult to treat. In the advanced setting, systemic treatments may be delivered as primary therapy before surgery or definitive radiotherapy, as exclusive treatment when no focal treatment is feasible, or in the setting of recurrences. Réseau tumeurs THYMIques et Cancer (RYTHMIC) is the nationwide network for TETs in France. The objective of the study was to describe the modalities and analyze the efficacy of systemic treatments for patients with advanced TETs included in the RYTHMIC prospective database hosted by the French Thoracic Cancer Intergroup. Methods: All consecutive patients for whom systemic treatment was discussed at the RYTHMIC multidisciplinary tumor board from 2012 to 2015 and who received at least one cycle of treatment were included. The main end points were objective response and progression‐free survival (PFS). Results: A total of 236 patients were included in this analysis. Of those 236 patients, 91 received primary chemotherapy, leading to response rates of 83% for thymomas and 75% for thymic carcinomas and a median PFS of 23.2 months. A strong predictor of longer PFS was histologic type of thymoma (p < 0.001). Exclusive chemotherapy was delivered to 54 patients. The response rates were 31% for thymomas and 37% for thymic carcinomas. The median PFS was 6.2 months, and it was correlated to response rate (p = 0.001). Systemic therapy for a first, second, third, and fourth recurrence was delivered to 114, 81, 51, and 27 patients, respectively. The response rates ranged between 15% and 39% for thymomas and 4% to 21% for thymic carcinomas. The median PFS times were 7.7, 6.2, 5.9, and 6.5 months, respectively. Conclusion: Patients with advanced thymic malignancies may receive multiple lines of systemic therapy, with an opportunity for clinically relevant PFS rates for which objective response may be a surrogate. Our real‐life study provides landmark efficacy data that are needed when designing clinical trials to assess innovative agents.

中文翻译：

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晚期胸腺上皮肿瘤的全身治疗：来自 RYTHMIC 前瞻性队列的见解

简介：胸腺上皮肿瘤 (TET) 是一种罕见的恶性肿瘤，可能具有侵袭性且难以治疗。在晚期情况下，全身治疗可作为手术前的主要治疗或根治性放疗，在没有局部治疗可行时或在复发情况下作为排他性治疗。Réseau tumeurs THYMIques et Cancer (RYTHMIC) 是法国 TET 的全国网络。该研究的目的是描述模式和分析系统治疗对晚期 TET 患者的疗效，该患者包括在法国胸癌组间组织托管的 RYTHMIC 前瞻性数据库中。方法：纳入 2012 年至 2015 年在 RYTHMIC 多学科肿瘤委员会讨论全身治疗并接受至少一个周期治疗的所有连续患者。主要终点是客观反应和无进展生存期（PFS）。结果：本次分析共纳入 236 名患者。在这 236 名患者中，91 名接受了初步化疗，导致胸腺瘤的缓解率为 83%，胸腺癌的缓解率为 75%，中位 PFS 为 23.2 个月。较长 PFS 的一个强预测因子是胸腺瘤的组织学类型 (p < 0.001)。54 名患者接受了独家化疗。胸腺瘤的反应率为 31%，胸腺癌的反应率为 37%。中位 PFS 为 6.2 个月，与缓解率相关 (p = 0.001)。分别对 114、81、51 和 27 名患者进行了针对第一次、第二次、第三次和第四次复发的全身治疗。胸腺瘤的反应率在 15% 到 39% 之间，胸腺癌的反应率在 4% 到 21% 之间。中位 PFS 时间分别为 7.7、6.2、分别为 5.9 和 6.5 个月。结论：晚期胸腺恶性肿瘤患者可能接受多线全身治疗，有机会获得临床相关的 PFS 率，客观反应可能是替代指标。我们的真实研究提供了设计临床试验以评估创新药物时所需的里程碑式疗效数据。