Department of Radiation Oncology, Institut Claudius Regaud, Institut Universitaire du, Cancer de Toulouse, Oncopole, Toulouse, France.
Department of Radiation Oncology, CHU La Timone, Marseille, France.
Int J Radiat Oncol Biol Phys. 2018 Sep 1;102(1):166-173. doi: 10.1016/j.ijrobp.2018.05.036. Epub 2018 May 24.
The objective of this study was to analyze survival and prognostic factors for children, adolescents, and young adults treated with postoperative radiation therapy (RT) for intracranial ependymoma.
Between 2000 and 2013, 202 patients aged ≤25 years were treated in the 13 main French pediatric RT reference centers. Their medical records were reviewed for information, treatments received, and survival rates. All children had received postoperative RT- conformal, intensity modulated, or proton beam. In 2009, the prescribed standard dose in France rose from 54 Gy to 59.4 Gy.
Median follow-up was 53.8 months (95% confidence interval [CI] 47-63.5). Median age at RT was 5 years (range 1-22), and 32% of the children treated were aged <3 years. Regarding treatment, 85.6% of patients underwent gross total resection, 62% of patients received conformal RT (vs 29% for intensity modulated RT and 8% for proton beam RT), 62.4% of patients received a dose >54 Gy, and 71% received chemotherapy. Of the 84 relapses, 75% were local. The cumulative incidence of local relapse was 24.4% (95% CI 18.2-31.2) at 3 years and 31.3% (95% CI 24-38.9) at 5 years. The 5-year disease-free survival (DFS) and overall survival rates were 50.4% (95% CI 42.2-58) and 71.4% (95% CI 63.1-78.2). Tumor grade was the only prognostic factor for local relapse and DFS. Tumor grade, age, and extent of resection were independent prognostic factors for overall survival.
We confirmed several clinical and tumoral prognostic factors in a large French multicenter study. DFS for intracranial ependymoma remains low, and new biological and imaging markers are needed to distinguish among different subtypes, adapt treatments, and improve survival.
本研究旨在分析接受术后放疗（RT）治疗的颅内室管膜瘤患儿、青少年和年轻成人的生存和预后因素。
在 2000 年至 2013 年间，202 名年龄≤25 岁的患者在 13 个法国主要儿科 RT 参考中心接受治疗。对其病历进行了回顾，以获取信息、治疗方法和生存率。所有患儿均接受了术后 RT-适形、调强或质子束治疗。2009 年，法国的标准剂量从 54 Gy 提高到 59.4 Gy。
中位随访时间为 53.8 个月（95%置信区间[CI] 47-63.5）。RT 时的中位年龄为 5 岁（范围 1-22），接受治疗的患儿中有 32%年龄<3 岁。关于治疗，85.6%的患者行大体全切除，62%的患者接受适形 RT（调强 RT 为 29%，质子束 RT 为 8%），62.4%的患者接受剂量>54 Gy，71%的患者接受化疗。84 例复发中，75%为局部复发。3 年时局部复发累积发生率为 24.4%（95%CI 18.2-31.2），5 年时为 31.3%（95%CI 24-38.9）。5 年无病生存率（DFS）和总生存率分别为 50.4%（95%CI 42.2-58）和 71.4%（95%CI 63.1-78.2）。肿瘤分级是局部复发和 DFS 的唯一预后因素。肿瘤分级、年龄和切除范围是总生存率的独立预后因素。
我们在一项大型法国多中心研究中证实了一些临床和肿瘤预后因素。颅内室管膜瘤的 DFS 仍然较低，需要新的生物学和影像学标志物来区分不同亚型，调整治疗方法，提高生存率。
