对视网膜母细胞瘤 (RB) 流行病学的研究可以改善治疗。旨在估计欧洲儿童中 RB 的发病率和生存率以及这些患者中第二原发肿瘤（RB 除外）的发生率。这项队列研究采用基于人群的方法数据来自 31 个欧洲国家的 81 个癌症登记处，遵循欧洲癌症登记处 (EUROCARE-6) 项目。数据收集于 2000 年 1 月至 2013 年 12 月期间进行。包括诊断为 RB 的 0 至 14 岁欧洲儿童。数据分析时间为 2023 年 5 月至 11 月。根据国际肿瘤疾病分类第三版 (ICD-O-3) 诊断 RB，形态编码 9510-9514（视网膜母细胞瘤）和恶性行为（形态编码第五位数字，3） .年发病率（每百万 0-14 岁儿童）、5 年生存率 (%) 以及随后恶性肿瘤的标准化发病率 (SIR)。该研究包括 3262 名患者（平均 [SD] 年龄，1.27 [1.63] ] 年；来自 81 个登记处的 1706 名男性（52%）和 1556 名女性（48%）。其中，排除时间覆盖不完整的登记后，将 3098 名患者纳入趋势分析：2000 年至 2003 年 940 名，2004 年至 2006 年 703 名，2007 年至 2009 年 744 名，2010 年至 2013 年 856 名。估计的欧洲总体发病率为4.0（95% CI，3.9-4.1）。各国的发病率从每年不到 200 万到超过 600 万不等。在任何地区均未观察到发病率的时间趋势。欧洲总体 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%），而一些国家的五年生存率为 100%。在随访期间（长达 14 年）记录了 25 例后续恶性肿瘤，SIR 为 8.2，不同部位病例的平均年龄在 1.3 至 8.9 岁之间。发现血液肿瘤（SIR，5）以及骨和软组织肉瘤（SIR，29）的风险增加。这项研究表明，从 2000 年到 2013 年，RB 发病率稳定在每 100 万名 0 至 14 岁欧洲儿童 4.0 例。但各国的估计各不相同，而且各国的生存率差异仍然存在。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，未来的登记研究应旨在收集诊断和治疗时的标准化 RB 阶段，以解释差异并可能改善监测。

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.