欧洲视网膜母细胞瘤儿童的生存和医疗保健负担

关于视网膜母细胞瘤（RB）流行病学的研究可能会改善管理。估计欧洲儿童RB的发病率和存活以及这些患者的第二个原发性肿瘤（除RB）的发生。这些同龄人使用的研究使用了31个欧洲国家对欧洲癌症注册的81个癌症注册者（Eurocare-6）的欧洲癌症中的81个癌症注册者（Eurocare-6）。数据收集发生在2000年1月至2013年12月之间。包括0至14岁的RB的欧洲儿童。分析数据从2023年5月至2023年11月进行分析。RB的诊断与国际肿瘤学疾病分类，第三版（ICD-O-3），编码为9510-9514（视网膜母细胞瘤）和恶性行为和恶性行为（第五位数字数字，形态代码，3）。该研究包括3262例患者（平均[SD]年龄，1.27 [1.63]年； 1706 [52％]男性和1556 [48％]女性来自81名注册表。其中，在排除时间不完全覆盖的注册表后，考虑了3098例患者：2000年至2003年的940例，2004年至2006年的703例，2007年至2009年的744例，2010年至2013年的856个。估计的欧洲总发病率为4.0（95％CI，3.9-9-4.1）。国家之间的比率从每年不到200万至600万。在任何区域均未观察到发病率的时间趋势。欧洲总5年生存率为97.8％（95％CI，95.5-98.9； 3180例）。爱沙尼亚和保加利亚的五年生存率较低（<80％），几个国家的生存率较低。在随访期间（长达14年）记录了25个随后的恶性肿瘤，SIR为8.2，案例发生在不同地点的平均年龄在1.3至8.9年之间。发现血液学肿瘤（SIR，5）和骨和软组织肉瘤的风险增加（SIR，29）。这项研究表明，RB发病率在2000年至2013年的0至14岁的每1000000名欧洲儿童中保持稳定，但估计在各国之间的估计差异差异。这些数据可用于监测RB管理和第二肿瘤的发生。研究结果表明，未来的注册表研究应旨在在诊断和治疗时收集标准化的RB阶段，以解释差异并有可能改善监视。

Studies on the epidemiology of retinoblastoma (RB) could lead to improvement in management.To estimate the incidence and survival of RB in European children and the occurrence of second primary tumors (other than RB) in these patients.This cohort study used population-based data from 81 cancer registries in 31 European countries adhering to the European Cancer Registries (EUROCARE-6) project. Data collection took place between January 2000 and December 2013. European children aged 0 to 14 years diagnosed with RB were included. Data were analyzed from May to November 2023.Diagnosis of RB with International Classification of Diseases for Oncology, Third Edition (ICD-O-3), morphology coded 9510-9514 (retinoblastoma) and malignant behavior (fifth digit of morphology code, 3).Annual incidence (per million children aged 0-14 years), 5-year survival (%), and the standardized incidence ratio (SIR) of subsequent malignant neoplasms.The study included 3262 patients (mean [SD] age, 1.27 [1.63] years; 1706 [52%] male and 1556 [48%] female) from 81 registries. Of these, 3098 patients were considered in trend analysis after excluding registries with incomplete time coverage: 940 in 2000 to 2003, 703 in 2004 to 2006, 744 in 2007 to 2009, and 856 in 2010 to 2013. The estimated overall European incidence rate was 4.0 (95% CI, 3.9-4.1). Rates among countries varied from less than 2 million to greater than 6 million per year. No time trend of incidence was observed in any area. The overall European 5-year survival was 97.8% (95% CI, 95.5-98.9; 3180 cases). Five-year survival was lower in Estonia and Bulgaria (<80%) and 100% in several countries. Twenty-five subsequent malignant neoplasms were recorded during follow-up (up to 14 years), with an SIR of 8.2 and with cases occurring at mean ages between 1.3 and 8.9 years across different sites. An increased risk was found for hematological tumors (SIR, 5) and bone and soft tissue sarcomas (SIR, 29).This study showed RB incidence remained stable at 4.0 per 1 000 000 European children aged 0 to 14 years from 2000 to 2013, but estimates varied among countries and differences in survival across countries persist. These data might be used to monitor RB management and occurrences of second tumors. The findings suggest future registry studies should aim to collect standardized RB stage at diagnosis and treatment to interpret disparities and potentially improve surveillance.