Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Br J Ophthalmol. 2021 Jan;105(1):63-69. doi: 10.1136/bjophthalmol-2019-315744. Epub 2020 Mar 25.
To determine the incidence of retinoblastoma (Rb) and subsequent survival in the Finnish population during five decades.
This retrospective observational cohort study comprised all patients with Rb born in Finland during 19642014 and diagnosed in 2018 (birth cohort analysis) or diagnosed in 19642014 (standard annual analysis), identified from the Finnish Cancer Registry and the national referral centre. We report age-adjusted incidences and survival according to cause of death.
Of children born in 19642014, 205 developed Rb, whereas 204 Rbs were diagnosed during these years; 196 belonged to both cohorts. Altogether 80 (38%) of the 213 children had heritable Rb and 19 (9%) had familial disease. The sex ratio was 1.34, suggesting male preponderance. Birth cohort analysis showed a median incidence of 6.2 per 100 000 live births (1:16 130) and less variability as compared with standard annual analysis (12.1, 6.5 and 4.4 per million children 04, 09 and 014 years of age, respectively). The incidence of heritable Rb increased with time, reflecting the increase in familial tumours. Five-year mortality rates from Rb were 6.2% and 7.6% for non-heritable and heritable diseases, respectively, and 35-year mortality rates from second malignancies were 0% and 14.3%, respectively. Family history predicted improved survival, whereas the period of diagnosis did not.
The incidence of familial Rb has increased, along with improvement in survival in Finland in 1964-2014, whereas the overall incidence of Rb was stable. Long-term risk of dying of second malignancies after heritable Rb was in line with other countries.
确定芬兰人群在五十年间视网膜母细胞瘤（Rb）的发病率和随后的生存率。
本回顾性观察性队列研究包括 1964 年至 2014 年期间在芬兰出生且于 2018 年诊断（出生队列分析）或于 1964 年至 2014 年期间诊断（标准年度分析）的所有 Rb 患者，这些患者均来自芬兰癌症登记处和国家转诊中心。我们根据死因报告了年龄调整后的发病率和生存率。
在 1964 年至 2014 年出生的儿童中，有 205 例发生 Rb，而这些年期间共诊断出 204 例 Rb；其中 196 例同时属于两个队列。共有 80 例（38%）儿童患有遗传性 Rb，19 例（9%）患有家族性疾病。男女比例为 1.34，提示男性占优势。出生队列分析显示，每 100000 例活产儿中有 6.2 例（1：16130）的中位发病率，与标准年度分析相比，变异度更小（分别为每百万儿童 04、09 和 014 岁时的 12.1、6.5 和 4.4 例）。遗传性 Rb 的发病率随时间增加，反映出家族性肿瘤的增加。非遗传性和遗传性疾病的 5 年 Rb 死亡率分别为 6.2%和 7.6%，遗传性 Rb 后 35 年第二恶性肿瘤的死亡率分别为 0%和 14.3%。家族史预测生存改善，而诊断时期则没有。
1964 年至 2014 年期间，芬兰遗传性 Rb 的发病率增加，生存率提高，而 Rb 的总体发病率保持稳定。遗传性 Rb 后长期死于第二恶性肿瘤的风险与其他国家一致。
