Central Brain Tumor Registry of the United States (CBTRUS), Hinsdale, IL, USA.
Department of Neurosurgery, Duke University School of Medicine, Durham, NC, USA.
J Neurooncol. 2023 Nov;165(2):279-290. doi: 10.1007/s11060-023-04480-7. Epub 2023 Nov 19.
Incidence, prevalence, and survival are population-based statistics describing cancer burden. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) specializes in tumor biology and outcomes for 12 rare CNS tumor types selected for their importance in adults, research interest, or potential for targeted treatment. The aim of this study was to update incidence, prevalence, and survival statistics for these tumors.
The Central Brain Tumor Registry of the United States (CBTRUS) database, a combined dataset of Centers for Disease Control and Prevention's (CDC) National Program of Cancer Registries (NPCR) and NCI's Surveillance, Epidemiology and End Results (SEER) data, was used to calculate average annual age-adjusted incidence rates (AAAIR) per 100,000 population overall and by sex, race-ethnicity, and age for diagnosis years 2008-2019. Incidence time trends were calculated for diagnosis years 2004-2019. NPCR data were used to calculate relative survival rates. Point prevalence on December 31, 2019 was estimated using annual age-specific incidence and survival.
AAAIR was 1.47 per 100,000 for these tumors combined, with highest incidence in ependymomas (AAAIR = 0.41/100,000). Most tumor types were more common in males, adults (ages 40 + years) or children (ages < 15 years), and non-Hispanic White individuals. Ependymomas were the most prevalent tumor type (19,320 cases) followed by oligodendrogliomas (14,900 cases). Ependymomas had the highest five-year survival (90.6%) and primary CNS sarcomas the lowest (7.7%).
These data provide means to measure the impact of clinical care and evaluate new therapies and the evolving histopathology definitions in rare CNS tumor types.
发病率、患病率和生存率是描述癌症负担的基于人群的统计数据。美国国家癌症研究所（NCI）综合肿瘤网络评估罕见中枢神经系统肿瘤（NCI-CONNECT）专注于肿瘤生物学和 12 种为成人选择的罕见中枢神经系统肿瘤类型的结果，这些肿瘤对研究感兴趣或具有潜在的靶向治疗。本研究的目的是更新这些肿瘤的发病率、患病率和生存率统计数据。
使用美国中央脑肿瘤登记处（CBTRUS）数据库，该数据库是疾病控制和预防中心（CDC）国家癌症登记处（NPCR）和 NCI 的监测、流行病学和最终结果（SEER）数据的综合数据集，计算 2008-2019 年诊断年份的每 10 万人年龄调整后的平均年发病率（AAAIR），总体和按性别、种族-族裔和年龄划分。计算了 2004-2019 年诊断年份的发病率时间趋势。使用 NPCR 数据计算相对生存率。使用每年特定年龄的发病率和生存率估计 2019 年 12 月 31 日的点患病率。
这些肿瘤的 AAAIR 合计为 1.47/100,000，其中室管膜瘤的发病率最高（AAAIR=0.41/100,000）。大多数肿瘤类型在男性、成年人（40 岁及以上）或儿童（<15 岁）和非西班牙裔白人中更为常见。室管膜瘤是最常见的肿瘤类型（19320 例），其次是少突胶质细胞瘤（14900 例）。室管膜瘤的五年生存率最高（90.6%），原发性中枢神经系统肉瘤最低（7.7%）。
这些数据提供了衡量临床护理影响的方法，并评估了罕见中枢神经系统肿瘤类型中新兴的组织病理学定义和新疗法的效果。
