National Cancer Registration and Analysis Service, Public Health England, 4150 Chancellor Court, Oxford Business Park South, Oxford, OX4 2GX, UK.
Histopathology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
BMC Cancer. 2019 Feb 11;19(1):139. doi: 10.1186/s12885-019-5344-7.
Intracranial and intraspinal tumours are the most numerous solid tumours in children. Some recently defined subtypes are relatively frequent in childhood. Many cancer registries routinely ascertain CNS tumours of all behaviours, while others only cover malignant neoplasms. Some behaviour codes have changed between revisions of the International Classification of Diseases for Oncology, including pilocytic astrocytoma, downgraded to uncertain behaviour in ICD-O-3.
We used data from the population-based National Registry of Childhood Tumours, which routinely included non-malignant CNS tumours, to document the occurrence of CNS tumours among children aged < 15 years in Great Britain during 2001-2010 and to document the descriptive epidemiology of childhood CNS tumours over the 40-year period 1971-2010, during which several new entities were accommodated in successive editions of the WHO Classification and revisions of ICD-O. Eligible cases were all those with a diagnosis included in Groups III (CNS tumours) and Xa (CNS germ-cell tumours) of the International Classification of Childhood Cancer, Third Edition. The population at risk was derived from annual mid-year estimates by sex and single year of age compiled by the Office for National Statistics and its predecessors. Incidence rates were calculated for age groups 0, 1-4, 5-9 and 10-14 years, and age-standardised rates were calculated using the weights of the world standard population.
Age-standardised incidence in 2001-10 was 40.1 per million. Astrocytomas accounted for 41%, embryonal tumours for 17%, other gliomas for 10%, ependymomas for 7%, rarer subtypes for 20% and unspecified tumours for 5%. Incidence of tumours classified as malignant and non-malignant by ICD-O-3 increased by 30 and 137% respectively between 1971-75 and 2006-10.
Total incidence was similar to that in other large western countries. Deficits of some, predominantly low-grade, tumours or differences in their age distribution compared with the United States and Nordic countries are compatible with delayed diagnosis. Complete registration regardless of tumour behaviour is essential for assessing burden of disease and changes over time. This is particularly important for pilocytic astrocytoma, because of its recent downgrading to non-malignant and time trends in the proportion of astrocytomas with specified subtype.
颅内和脊髓肿瘤是儿童中最常见的实体肿瘤。一些最近定义的亚型在儿童中相对常见。许多癌症登记处通常确定所有行为的中枢神经系统肿瘤，而其他登记处仅覆盖恶性肿瘤。国际肿瘤学疾病分类的修订版之间，一些行为代码发生了变化，包括毛细胞星形细胞瘤，在 ICD-O-3 中降级为不确定行为。
我们使用基于人群的英国国家儿童肿瘤登记处的数据，该登记处通常包括非恶性中枢神经系统肿瘤，记录了 2001-2010 年期间英国 15 岁以下儿童中枢神经系统肿瘤的发生情况，并记录了 1971-2010 年 40 年间儿童中枢神经系统肿瘤的描述性流行病学，在此期间，一些新实体在世界卫生组织分类的连续版本和 ICD-O 的修订版中得到了包含。合格病例为国际儿童癌症分类第三版第 III 组（中枢神经系统肿瘤）和 Xa 组（中枢神经系统生殖细胞肿瘤）中包含的所有诊断病例。风险人群来源于英国国家统计局及其前身每年按性别和年龄编制的年中估计数。计算了 0、1-4、5-9 和 10-14 岁年龄组的发病率，并使用世界标准人口的权重计算了年龄标准化率。
2001-10 年的年龄标准化发病率为 40.1/100 万。星形细胞瘤占 41%，胚胎肿瘤占 17%，其他神经胶质瘤占 10%，室管膜瘤占 7%，罕见亚型占 20%，未特指肿瘤占 5%。ICD-O-3 分类为恶性和非恶性的肿瘤发病率分别在 1971-75 年和 2006-10 年之间增加了 30%和 137%。
总发病率与其他西方国家相似。与美国和北欧国家相比，某些肿瘤（主要是低级别肿瘤）的发病率较低或其年龄分布存在差异，这与诊断延迟有关。无论肿瘤行为如何，完整的登记对于评估疾病负担和随时间的变化都是必不可少的。对于毛细胞星形细胞瘤来说，这一点尤为重要，因为它最近被降级为非恶性肿瘤，而且星形细胞瘤中具有特定亚型的比例也存在时间趋势。
