确定 12 种选定的罕见中枢神经系统肿瘤影响的合作伙伴关系：来自 CBTRUS 和 NCI-CONNECT 的报告。
Partnership for defining the impact of 12 selected rare CNS tumors: a report from the CBTRUS and the NCI-CONNECT.
机构信息
Central Brain Tumor Registry of the United States, Hinsdale, IL, USA.
Case Comprehensive Cancer Center, Cleveland, OH, USA.
出版信息
J Neurooncol. 2019 Aug;144(1):53-63. doi: 10.1007/s11060-019-03215-x. Epub 2019 Jun 17.
PURPOSE
Population-based cancer statistics, including histology-specific incidence, prevalence, and survival are essential to evaluating the total burden due to disease in a population. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) was developed to better understand tumor biology and patient outcomes for 12 selected brain and other central nervous system (CNS) tumor histologies that are rare in adults to improve approaches to care and treatment. The aim of this study was to determine the incidence, prevalence, and survival of these selected rare histologies.
METHODS
Data from the Central Brain Tumor Registry of the United States (CBTRUS) from 2000 to 2014 were used to calculate average annual age-adjusted incidence rates (AAIR) per 100,000 population overall and by sex, race, ethnicity, and age. NCI's Surveillance, Epidemiology and End Results (SEER) data were used to calculate relative survival (RS) estimates. Point prevalence for 2014 was estimated using annual age-specific incidence and survival from CBTRUS and SEER, respectively.
RESULTS
Overall AAIR was 1.47 per 100,000 for all 12 rare histologies combined, with the highest histology-specific incidence in oligodendrogliomas (AAIR = 0.40/100,000). Overall, most histologies were more common in males, adults (age 40 + ), Whites, and non-Hispanics. Ependymomas were the most prevalent histology at 4.11 per 100,000; followed by oligodendrogliomas at 3.68 per 100,000. Relative survival at 1-, 5-, and 10-years was 82.3%, 64.0%, and 55.4%, respectively for all 12 selected brain and other CNS tumor types combined. Ependymomas had the highest RS (1-year = 94.2%, 5-year = 83.9%, 10-year = 78.6%) and gliosarcomas had the lowest relative survival rate (1-year = 42.5%, 5-year = 5.6%, 10-year = 2.9%) at all three time points.
CONCLUSIONS
Incidence and prevalence of these rare brain and other CNS tumor histologies have not been previously reported. Along with survival, these data provide a statistical foundation to understand the impact of these cancers and provide important disease-specific data for the design of prospective clinical trials.
目的
基于人群的癌症统计数据，包括组织学特异性发病率、患病率和生存率，对于评估人群中疾病的总负担至关重要。美国国家癌症研究所（NCI）的综合肿瘤网络评估罕见中枢神经系统（CNS）肿瘤（NCI-CONNECT）旨在更好地了解 12 种选定的脑和其他中枢神经系统（CNS）肿瘤组织学的肿瘤生物学和患者结局，这些组织学在成年人中较为罕见，以改善治疗方法。本研究的目的是确定这些选定的罕见组织学的发病率、患病率和生存率。
方法
使用美国中枢神经系统肿瘤登记处（CBTRUS）的数据，计算了 2000 年至 2014 年期间所有 12 种罕见组织学的平均年龄调整发病率（AAIR），以每 10 万人为单位，按性别、种族、族裔和年龄进行计算。利用 NCI 的监测、流行病学和最终结果（SEER）数据计算相对生存率（RS）估计值。使用 CBTRUS 和 SEER 的年度年龄特异性发病率和生存率分别估算 2014 年的点患病率。
结果
所有 12 种罕见组织学的总体 AAIR 为 1.47/10 万，其中少突胶质细胞瘤的组织学特异性发病率最高（AAIR=0.40/10 万）。总体而言，大多数组织学在男性、成年人（40 岁+）、白人和非西班牙裔人群中更为常见。室管膜瘤的患病率最高，为每 10 万人 4.11 例；其次是少突胶质细胞瘤，每 10 万人 3.68 例。所有 12 种选定的脑和其他 CNS 肿瘤类型的 1 年、5 年和 10 年相对生存率分别为 82.3%、64.0%和 55.4%。室管膜瘤的 RS 最高（1 年=94.2%，5 年=83.9%，10 年=78.6%），而神经胶质瘤肉瘤的相对生存率最低（1 年=42.5%，5 年=5.6%，10 年=2.9%）。
结论
这些罕见的脑和其他 CNS 肿瘤组织学的发病率和患病率以前没有报道过。结合生存率，这些数据为了解这些癌症的影响提供了统计学基础，并为前瞻性临床试验的设计提供了重要的疾病特异性数据。
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