背景:很少有研究对儿童室管膜瘤幸存者的后期效应负担进行表征。为了解决这个差距,我们在基于人群的室管膜瘤幸存者队列中使用真实世界的卫生服务数据检查了这些后遗症.
方法:安大略省所有18岁以下被诊断为室管膜瘤的患者,加拿大在1987年至2015年期间,他们从最近的儿科癌症事件(索引日期)中存活了至少5年,与人口对照组1:5相匹配。根据与省级卫生服务数据的联系,比较了幸存者和对照组之间多种医学结局和功能结局的累积发生率.
结果:在96名幸存者中,77.1%接受了照射,9.4%接受了顺铂治疗。在指数发布后的10年,幸存者的全因死亡率风险明显较高(7.1%,95%置信区间[CI]:1.0-13.3vs.0.3%,95%CI:0.0-1.0;p=.0002),非产科住院(45.1%,95%CI:32.6-56.7vs.10.6%,95%CI:7.6-14.1;p<.0001),中风(6.5%,95%CI:2.3-13.7vs.0%;p<.0001),需要放大装置的严重听力损失(7.5%,95%CI:2.7-15.7vs.0%;p<.0001),接受家庭护理服务(27.6%,95%CI:18.5-37.5vs.7.7%,95%CI:5.3-10.7;p<.0001),并提交残疾支持处方索赔(24.0%,95%CI:14.8-34.3vs.5.4%,95%CI:3.5-7.8;p<0.0001)与对照组相比。
结论:儿科室管膜瘤幸存者极易出现严重的晚期后遗症,包括死亡,中风,严重的听力损失,和残疾。迫切需要努力改善风险分层方法,以减轻风险较低疾病儿童的毒性疗法暴露。预防或降低晚期后遗症风险的干预措施对于优化幸存者的长期健康至关重要。
BACKGROUND: Few studies have characterized the burden of late effects among childhood
ependymoma survivors. To address this gap, we examined these sequelae using real-world health services data in a population-based
ependymoma survivor cohort.
METHODS: All individuals younger than 18 years diagnosed with an
ependymoma in Ontario, Canada between 1987 and 2015 who had survived at least 5 years from their latest pediatric cancer event (index date) were matched 1:5 with population controls. Following linkage with provincial health services data, the cumulative incidences of multiple medical and functional outcomes between survivors and controls were compared.
RESULTS: Among 96 survivors, 77.1% had been irradiated and 9.4% had received cisplatin. At 10 years post-index, survivors were at significantly higher risk of all-cause mortality (7.1%, 95% confidence interval [CI]: 1.0-13.3 vs. 0.3%, 95% CI: 0.0-1.0; p = .0002), non-obstetric hospitalization (45.1%, 95% CI: 32.6-56.7 vs. 10.6%, 95% CI: 7.6-14.1; p < .0001), stroke (6.5%, 95% CI: 2.3-13.7 vs. 0%; p < .0001), severe hearing loss requiring an amplification device (7.5%, 95% CI: 2.7-15.7 vs. 0%; p < .0001), receiving homecare service (27.6%, 95% CI: 18.5-37.5 vs. 7.7%, 95% CI: 5.3-10.7; p < .0001), and submitting a disability support prescription claim (24.0%, 95% CI: 14.8-34.3 vs. 5.4%, 95% CI: 3.5-7.8; p < .0001) compared to controls.
CONCLUSIONS: Pediatric
ependymoma survivors are highly vulnerable to severe late sequelae, including death, stroke, severe hearing loss, and disability. Urgent efforts are needed to improve risk-stratification approaches that mitigate exposure to toxic therapies for children with lower risk disease. Interventions to prevent or decrease the risk of developing late sequelae are critical to optimizing survivor long-term health.