Department of Radiotherapy and Radiooncology, University Hospital of Muenster, Muenster, Germany.
Pediatrics III, Hematology/ Oncology, West German Cancer Center and German Cancer Research Center, University Hospital Essen, Essen, Germany.
Int J Radiat Oncol Biol Phys. 2018 Nov 1;102(3):584-592. doi: 10.1016/j.ijrobp.2018.06.032. Epub 2018 Jul 3.
There is no standard treatment procedure for relapsed Ewing sarcoma (EwS). This retrospective analysis evaluates the survival outcome in patients with an isolated pulmonary relapse of EwS treated with whole lung irradiation (WLI) in addition to second line chemotherapy (Ctx).
In our study, 136 patients with pulmonary relapsed EwS who were registered in the relapse register of the Cooperative Ewing Sarcoma Study group or the Sarcoma Relapse Registry for relapsed sarcoma of bone and soft tissues were analyzed. All patients received relapse Ctx or an additional total resection of lung metastasis. Of these patients, 88 (median age, 21 years; range, 7-52 years) achieved a second remission by the relapse treatment. Of these 88 patients, 48 patients received an additional WLI. The 3-year progression-free survival (PFS) and 3-year overall survival (OS) were analyzed (median follow-up, 3 years; range, 7 months to 11 years and 9 months). Additional prognostic factors for survival outcomes, including the response of lung metastases to Ctx, were also estimated.
The survival outcome was significantly improved after WLI when analyzing the entire group of pulmonary relapsed patients: 3-year PFS 36% (+WLI) versus 14% (-WLI) (P = .001); 3- year OS 47% (+WLI) versus 33% (-WLI) (P = .007). The 3-year PFS in patients with complete remission of lung relapse receiving WLI (n = 48) compared with those without WLI (n = 40), was 37% (+WLI) versus 21% (-WLI) (P = .18). The site of the primary tumor and the response of pulmonary lesions to Ctx were significant prognostic indicators for survival in patients treated with WLI. No severe pulmonary function disorders or lung toxicities were observed after WLI treatment in both pediatric and adult patients.
The WLI does not correlate with improved OS in patients with pulmonary relapsed EwS. However, a marginal trend toward superior PFS and improved local control of pulmonary disease suggests the application of WLI in patients with EwS with isolated lung relapse and second clinical remission.
对于复发性尤因肉瘤(EwS),目前尚无标准的治疗方案。本回顾性分析评估了在接受全肺照射(WLI)联合二线化疗(Ctx)治疗孤立性肺复发 EwS 的患者的生存结局。
在我们的研究中,分析了登记在合作尤因肉瘤研究组复发登记处或骨骼和软组织肉瘤复发登记处的 136 例肺复发 EwS 患者。所有患者均接受复发 Ctx 或肺转移灶的额外完全切除术。在这些患者中,有 88 例(中位年龄 21 岁;范围为 7-52 岁)通过复发治疗获得第二次缓解。在这 88 例患者中,有 48 例接受了额外的 WLI。分析了 3 年无进展生存率(PFS)和 3 年总生存率(OS)(中位随访时间为 3 年;范围为 7 个月至 11 年和 9 个月)。还估计了包括肺转移灶对 Ctx 反应在内的其他生存预后因素。
在分析整个肺复发患者组时,WLI 后的生存结果显著改善:3 年 PFS 为 36%(+WLI)对比 14%(-WLI)(P=0.001);3 年 OS 为 47%(+WLI)对比 33%(-WLI)(P=0.007)。接受 WLI 治疗的肺复发完全缓解患者(n=48)与未接受 WLI 治疗的患者(n=40)相比,3 年 PFS 为 37%(+WLI)对比 21%(-WLI)(P=0.18)。原发肿瘤部位和肺部病变对 Ctx 的反应是接受 WLI 治疗的患者生存的重要预后指标。在儿童和成人患者中,WLI 治疗后均未观察到严重的肺功能障碍或肺毒性。
WLI 与肺复发 EwS 患者的 OS 改善无关。然而,PFS 略有改善且肺部疾病局部控制改善提示在孤立性肺复发且有第二次临床缓解的 EwS 患者中应用 WLI。