BACKGROUND Childhood cancers represent a small proportion of all cancers but are still a major public health problem. The study analysed long-term trends in childhood cancer incidence and survival in Estonia in relation to societal and health care transition. METHODS Data on all malignant tumours, diagnosed in children aged 0-14 during 1970-2016, were derived from the Estonian Cancer Registry. Age-standardised (World standard) incidence rates were calculated by ICCC-3 site groups and joinpoint regression was used to estimate annual percentage change (APC) for incidence trends. Cohort and period approach were used to estimate 5-year survival. Internal age standardisation was applied. RESULTS A total of 1628 incident cancer cases were diagnosed during the study period and overall incidence increased significantly at a rate of 0.5% per year. Significant increases were seen for neuroblastoma and germ cell tumours, for lymphoid leukemias and some CNS sub-sites. At the same time, decline in incidence was seen in almost all subgroups of unspecified neoplasms. The overall 5-year survival improved from 24% in 1970-1979 to 73% in 2010-2016, with the largest changes occurring in the 1990s and 2000s. For many sites, survival increase thereafter has been marginal. CONCLUSION In this first comprehensive population-based study of childhood cancer incidence and survival in Estonia, long-term trends are shown in the context of societal and health care changes. Even though the increasing incidence of some sites may, at least partially, be explained by improved diagnostics reflected in the decreased incidence of unspecified neoplasms, the overall cancer incidence in children seems to be rising. Rapid progress in diagnosis and care have improved childhood cancer survival immensely, but deficit in Estonia persists compared to other European countries. Results of the study accentuate the need for a more in-depth analysis of clinical data, but also for the prioritization of childhood cancer in Estonia, to ensure access to standard care and innovative treatments.

中文翻译：

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爱沙尼亚的儿童癌症发病率和生存趋势（1970-2016年）：一项基于人口的全国性研究。

背景技术儿童期癌症占所有癌症的一小部分，但仍然是主要的公共卫生问题。该研究分析了爱沙尼亚儿童癌症发病率和存活率与社会和医疗保健转型相关的长期趋势。方法1970-2016年期间诊断为0-14岁儿童的所有恶性肿瘤数据均来自爱沙尼亚癌症登记处。通过ICCC-3站点组计算了年龄标准化（世界标准）的发病率，并使用联合回归分析来估计发病趋势的年度百分比变化（APC）。队列和时期方法用于估计5年生存率。内部年龄标准化已应用。结果在研究期间共诊断出1628例癌症病例，总发病率以每年0.5％的速度显着增加。神经母细胞瘤和生殖细胞肿瘤，淋巴样白血病和某些中枢神经系统亚部位明显增加。同时，几乎所有未指明的肿瘤亚组的发病率均下降。总体5年生存率从1970-1979年的24％提高到2010-2016年的73％，变化最大的是1990年代和2000年代。对于许多地点而言，此后的生存率增长很小。结论在这项针对爱沙尼亚儿童癌症发病率和生存率的首次全面的基于人群的研究中，在社会和医疗保健变化的背景下显示了长期趋势。即使某些部位的发病率增加（至少部分地）可以通过未明确的肿瘤发病率降低反映出的诊断改善来解释，但儿童总体癌症发病率似乎正在上升。在诊断和护理方面的快速进步极大地改善了儿童期癌症的存活率，但与其他欧洲国家相比，爱沙尼亚的赤字持续存在。研究结果强调需要对临床数据进行更深入的分析，同时也需要优先考虑爱沙尼亚儿童期癌症，以确保获得标准治疗和创新治疗。