Long-Term Outcomes Among Adult Survivors of Childhood Central Nervous System Malignancies in the Childhood Cancer Survivor Study
Long-Term Outcomes Among Adult Survivors of Childhood Central Nervous System Malignancies in the Childhood Cancer Survivor Study
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DOI：
10.1093/jnci/djp148
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发表时间：
2009-07-01
影响因子：
10.3
通讯作者：
Packer, Roger J.
中科院分区：
文献类型：
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作者：
Armstrong, Gregory T.;Liu, Qi;Packer, Roger J.
儿童中枢神经系统（CNS）恶性肿瘤的成年幸存者面临长期发病和晚期死亡的高风险。然而，个体诊断和治疗组的晚期死亡模式、随后肿瘤和衰弱性医疗状况的长期风险以及社会人口学结果尚未得到全面表征。我们在儿童癌症幸存者研究中收集了 1970 年至 1986 年间诊断的中枢神经系统恶性肿瘤成人 5 年幸存者的治疗、死亡率、慢性医疗状况和神经认知功能的信息。使用竞争风险框架，我们根据死亡原因计算了累积死亡率，并根据颅脑放射治疗（RT）的暴露和剂量计算了后续肿瘤的累积发病率。根据对特定大脑区域的中枢神经系统放射治疗剂量来评估神经认知障碍和社会经济结果。使用 Cox 回归模型比较幸存者和兄弟姐妹之间慢性疾病的累积发生率。所有具有统计显着性的检验都是双向的。在所有符合条件的 5 年幸存者 (n = 2821) 中，30 年累积晚期死亡率为 25.8%（95% 置信区间 [CI] = 23.4% 至 28.3%），主要是由于原发性疾病的复发和/或进展。接受 50 Gy 或以上颅脑放疗的患者 (n = 813) 在诊断后 25 年时中枢神经系统内后续肿瘤的累积发生率为 7.1% (95% CI = 4.5% 至 9.6%)，而未接受放疗的患者为 1.0% (95% CI = 0% 至 2.3%)。幸存者在诊断后 5 年或更长时间内出现新的内分泌、神经或感觉并发症的风险高于兄弟姐妹。神经认知障碍很高，并且与特定肿瘤类型的辐射剂量成正比。额叶和/或颞叶放疗与较低的就业率和结婚率之间存在剂量依赖性关联。儿童中枢神经系统恶性肿瘤的幸存者晚期死亡以及随后发生肿瘤和慢性疾病的风险很高。应告知护理人员这些风险，以便他们能够提供风险导向的护理并制定筛查指南。
Adult survivors of childhood central nervous system (CNS) malignancies are at high risk for long-term morbidity and late mortality. However, patterns of late mortality, the long-term risks of subsequent neoplasms and debilitating medical conditions, and sociodemographic outcomes have not been comprehensively characterized for individual diagnostic and treatment groups.We collected information on treatment, mortality, chronic medical conditions, and neurocognitive functioning of adult 5-year survivors of CNS malignancies diagnosed between 1970 and 1986 within the Childhood Cancer Survivor Study. Using competing risk framework, we calculated cumulative mortality according to cause of death and cumulative incidence of subsequent neoplasms according to exposure and dose of cranial radiation therapy (RT). Neurocognitive impairment and socioeconomic outcomes were assessed with respect to dose of CNS radiotherapy to specific brain regions. Cumulative incidence of chronic medical conditions was compared between survivors and siblings using Cox regression models. All tests of statistical significance were two-sided.Among all eligible 5-year survivors (n = 2821), cumulative late mortality at 30 years was 25.8% (95% confidence interval [CI] = 23.4% to 28.3%), due primarily to recurrence and/or progression of primary disease. Patients who received cranial RT of 50 Gy or more (n = 813) had a cumulative incidence of a subsequent neoplasm within the CNS of 7.1% (95% CI = 4.5% to 9.6%) at 25 years from diagnosis compared with 1.0% (95% CI = 0% to 2.3%) for patients who had no RT. Survivors had higher risk than siblings of developing new endocrine, neurological, or sensory complications 5 or more years after diagnosis. Neurocognitive impairment was high and proportional to radiation dose for specific tumor types. There was a dose-dependent association between RT to the frontal and/or temporal lobes and lower rates of employment, and marriage.Survivors of childhood CNS malignancies are at high risk for late mortality and for developing subsequent neoplasms and chronic medical conditions. Care providers should be informed of these risks so they can provide risk-directed care and develop screening guidelines.