Achey Rebecca L, Vo Sierra, Cioffi Gino, Gittleman Haley, Schroer Julia, Khanna Vishesh, Buerki Robin, Kruchko Carol, Barnholtz-Sloan Jill S
Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, Ohio.
Department of Mathematics, Applied Mathematics, and Statistics, Case Western Reserve, Cleveland, Ohio.
Neurooncol Pract. 2020 May 9;7(5):549-558. doi: 10.1093/nop/npaa023. eCollection 2020 Oct.
Ependymoma is a rare CNS tumor arising from the ependymal lining of the ventricular system. General differences in incidence and survival have been noted but not examined on a comprehensive scale for all ages and by histology. Despite the rarity of ependymomas, morbidity/mortality associated with an ependymoma diagnosis justifies closer examination.
Incidence data were obtained from the Central Brain Tumor Registry of the United States in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, and survival data from Surveillance Epidemiology and End Results, from 2000 to 2016 for anaplastic ependymoma and ependymoma, not otherwise specified (NOS). Age-adjusted incidence rates (IRs) per 100 000 person-years were analyzed by age, sex, race, and location. Survival analysis was performed with Kaplan-Meier curves and multivariable Cox proportional hazards models.
Incidence of anaplastic ependymoma was highest in ages 0 to 4 years. African American populations had lower incidence but had a 78% increased risk of death compared to white populations (hazard ratio [HR]: 1.78 [95% CI, 1.30-2.44]). Incidence was highest for anaplastic ependymoma in the supratentorial region. Adults (age 40+ years) had almost twice the risk of death compared to children (ages 0-14 years) (HR: 1.97 [95% CI, 1.45-2.66]). For ependymoma, NOS, subtotal resection had a risk of mortality 1.86 times greater than gross total resection ([HR: 1.86 [95% CI, 1.32-2.63]).
African American populations experienced higher mortality rates despite lower incidence compared to white populations. Extent of resection is an important prognostic factor for survival. This highlights need for further evaluation of treatment patterns and racial disparities in the care of patients with ependymoma subtypes.
室管膜瘤是一种罕见的中枢神经系统肿瘤，起源于脑室系统的室管膜内衬。已注意到发病率和生存率的总体差异，但尚未针对所有年龄段并按组织学进行全面研究。尽管室管膜瘤罕见，但与室管膜瘤诊断相关的发病率/死亡率仍值得更深入研究。
发病率数据来自美国中央脑肿瘤登记处，与疾病控制和预防中心以及国家癌症研究所合作获取；生存数据来自监测、流行病学和最终结果数据库，涵盖2000年至2016年的间变性室管膜瘤和未另行指定（NOS）的室管膜瘤。按年龄、性别、种族和部位分析每10万人年的年龄调整发病率（IR）。采用Kaplan-Meier曲线和多变量Cox比例风险模型进行生存分析。
间变性室管膜瘤的发病率在0至4岁年龄组最高。非裔美国人的发病率较低，但与白人相比，死亡风险增加78%（风险比[HR]：1.78[95%置信区间，1.30 - 2.44]）。间变性室管膜瘤在幕上区域的发病率最高。与儿童（0 - 14岁）相比，成年人（40岁及以上）的死亡风险几乎高出一倍（HR：1.97[95%置信区间，1.45 - 2.66]）。对于未另行指定的室管膜瘤（NOS），次全切除的死亡风险比全切除高1.86倍（[HR：1.86[95%置信区间，1.32 - 2.63]）。
与白人相比，非裔美国人尽管发病率较低，但死亡率较高。切除范围是生存的重要预后因素。这突出表明需要进一步评估室管膜瘤亚型患者的治疗模式和种族差异。
