Background: Central nervous system (CNS) neoplasms are highly frequent solid tumours in children and adolescents. While some studies have shown a rise in their incidence in Europe, others have not. Survival remains limited. We addressed two questions about these tumours in Spain: (1) Is incidence increasing? and (2) Has survival improved? Methods: This population-based study included 1635 children and 328 adolescents from 11 population-based cancer registries with International Classification of Childhood Cancer Group III tumours, incident in 1983–2007. Age-specific and age-standardised (world population) incidence rates (ASRws) were calculated. Incidence time trends were characterised using annual percent change (APC) obtained with Joinpoint. Cases from 1991 to 2005 (1171) were included in Kaplan–Meier survival analyses, and the results were evaluated with log-rank and log-rank for trend tests. Children’s survival was age-standardised using: (1) the age distribution of cases and the corresponding trends assessed with Joinpoint; and (2) European weights for comparison with Europe. Results: ASRw 1983–2007: children: 32.7 cases/106; adolescents: 23.5 cases/106. The overall incidence of all tumours increased across 1983–2007 in children and adolescents. Considering change points, the APCs were: (1) children: 1983–1993, 4.3%^ (1.1; 7.7); 1993–2007, −0.2% (−1.9; 1.6); (2) adolescents: 1983–2004: 2.9%^ (0.9; 4.9); 2004–2007: −7.7% (−40; 41.9). For malignant tumours, the trends were not significant. 5-year survival was 65% (1991–2005), with no significant trends (except for non-malignant tumours). Conclusions: CNS tumour incidence in Spain was found to be similar to that in Europe. Rises in incidence may be mostly attributable to changes in the registration of non-malignant tumours. The overall malignant CNS tumour trend was compatible with reports for Southern Europe. Survival was lower than in Europe, without improvement over time. We provide a baseline for assessing current paediatric oncology achievements and incidence in respect of childhood and adolescent CNS tumours.
背景：中枢神经系统肿瘤是儿童和青少年中高发的实体肿瘤。尽管部分研究显示其在欧洲的发病率有所上升，但其他研究并未证实这一趋势。患者生存率仍有限。本研究针对西班牙中枢神经系统肿瘤提出两个问题：（1）发病率是否上升？（2）生存率是否改善？ 方法：这项基于人群的研究纳入了来自11个基于人群的癌症登记处的1635名儿童和328名青少年，这些病例均符合国际儿童癌症分类第三版标准，发病时间为1983年至2007年。计算了年龄别发病率和年龄标准化（世界人口）发病率。使用Joinpoint软件计算年度百分比变化以描述发病率的时间趋势。1991年至2005年的病例（1171例）纳入Kaplan-Meier生存分析，结果通过log-rank检验和趋势log-rank检验进行评估。儿童生存率采用以下方法进行年龄标准化：（1）病例的年龄分布及使用Joinpoint评估的相应趋势；（2）采用欧洲权重以便与欧洲数据进行比较。 结果：1983-2007年年龄标准化发病率：儿童：32.7例/10^6；青少年：23.5例/10^6。1983年至2007年间，儿童和青少年所有肿瘤的总体发病率均有所上升。考虑变化点后，年度百分比变化为：（1）儿童：1983-1993年，4.3%^（1.1；7.7）；1993-2007年，-0.2%（-1.9；1.6）；（2）青少年：1983-2004年：2.9%^（0.9；4.9）；2004-2007年：-7.7%（-40；41.9）。对于恶性肿瘤，趋势不显著。5年生存率为65%（1991-2005年），无显著趋势（非恶性肿瘤除外）。 结论：西班牙中枢神经系统肿瘤发病率与欧洲相似。发病率的上升可能主要归因于非恶性肿瘤登记方式的变化。总体恶性中枢神经系统肿瘤趋势与南欧报告相符。生存率低于欧洲水平，且随时间推移未见改善。本研究为评估当前儿科肿瘤学在儿童和青少年中枢神经系统肿瘤方面的成就和发病率提供了基线数据。
肿瘤(癌症)患者之家