Background/Objectives: Treatment of childhood ependymoma evolved from 1970 to 1999 by reducing radiation volumes and incorporating chemotherapy. The impact of these changes on long-term health outcomes remains unknown. In this report, we evaluated temporal changes in all-cause and cause-specific late mortality, chronic health conditions (CHCs), and subsequent neoplasms (SNs) in the Childhood Cancer Survivor Study (CCSS) cohort of adult survivors of pediatric ependymoma, diagnosed between 1970 and 1999. Methods: A total of 404 five-year survivors of ependymoma (47.5% female, 80.7% non-Hispanic White, median 6 (range 0–20) years at diagnosis, 22 (5–49) years from diagnosis) diagnosed between 1970 and 1999 and enrolled in the Childhood Cancer Survivor Study were evaluated for late (>5 years from diagnosis) mortality, SNs, and CHCs. Outcomes were analyzed by diagnosis decade, radiotherapy, and chemotherapy exposure. Gray’s test compared cumulative incidences. Multivariable piecewise exponential models estimated relative risks (RRs). Results: Whole-brain radiation exposure decreased over time (42.9% (1970s) to 2.7% (1990s)), while focal radiation (21.4% to 68.9%), and chemotherapy (29.5% to 50.2%) use increased. Fifteen-year all-cause late mortality (incidence, 95% CI) remained similar across decades: 1970s (9.3%, 3.4–18.8%), 1980s (14.7%, 9.4–21.2%), 1990s (10.3%, 6.7–14.9%). All-cause late mortality was higher after treatment with whole-brain radiation (22.5%, 11.2–36.5%) compared to focal radiation (11.4%, 7.5–16.1%) or no brain radiation (3.5%, 0.9–9.1%) (p< 0.001), and with chemotherapy (14.4%, 9.6–20.0%) versus without (6.8%, 3.8–11.0%) (p= 0.004). Compared to no brain radiation, the RR (95% CI) of grade 3–4 CHCs increased among survivors treated with focal (2.6, 1.3–5.4) and whole-brain radiation (3.5, 1.5–8.1), while chemotherapy was not associated with CHCs or SNs. Conclusions: Despite reduced radiation volumes and increased use of chemotherapy, late mortality and morbidity among pediatric ependymoma survivors remained largely unchanged across treatment decades.
背景/目的：从1970年至1999年，儿童室管膜瘤的治疗通过缩小放疗范围和引入化疗而不断发展。这些变化对长期健康结局的影响尚不明确。本研究基于儿童癌症幸存者研究队列，评估了1970年至1999年间确诊的儿童室管膜瘤成年幸存者的全因及特定原因远期死亡率、慢性健康状况和继发肿瘤随时间的变化趋势。方法：纳入1970年至1999年间确诊并参与儿童癌症幸存者研究的404例室管膜瘤五年生存者（女性47.5%，非西班牙裔白人80.7%，确诊中位年龄6岁[范围0-20岁]，确诊后随访中位时间22年[范围5-49年]），评估其远期（确诊5年后）死亡率、继发肿瘤及慢性健康状况。按确诊年代、放疗和化疗暴露情况分析结局指标，采用Gray检验比较累积发生率，通过多变量分段指数模型估算相对风险。结果：全脑放疗使用率随时间推移下降（1970年代42.9%→1990年代2.7%），而局部放疗（21.4%→68.9%）和化疗（29.5%→50.2%）使用率上升。各年代15年全因远期死亡率（发生率，95%置信区间）保持稳定：1970年代（9.3%，3.4-18.8%）、1980年代（14.7%，9.4-21.2%）、1990年代（10.3%，6.7-14.9%）。全脑放疗组的全因远期死亡率（22.5%，11.2-36.5%）显著高于局部放疗组（11.4%，7.5-16.1%）或无脑放疗组（3.5%，0.9-9.1%）（p<0.001）；化疗组（14.4%，9.6-20.0%）亦高于非化疗组（6.8%，3.8-11.0%）（p=0.004）。与无脑放疗相比，局部放疗（2.6，1.3-5.4）和全脑放疗（3.5，1.5-8.1）幸存者的3-4级慢性健康状况相对风险显著升高，而化疗与慢性健康状况或继发肿瘤无显著关联。结论：尽管放疗范围缩小且化疗使用增加，但儿童室管膜瘤幸存者的远期死亡率和发病率在不同治疗年代间基本保持稳定。
肿瘤(癌症)患者之家