Elsevier

European Journal of Cancer

Volume 42, Issue 13, September 2006, Pages 2064-2080
European Journal of Cancer

Childhood central nervous system tumours – incidence and survival in Europe (1978–1997): Report from Automated Childhood Cancer Information System project

https://doi.org/10.1016/j.ejca.2006.05.009Get rights and content

Abstract

This paper describes the incidence and survival of childhood central nervous system (CNS) tumours in Europe for the period 1978–1997. A total of 19,531 cases, aged 0–14 years, from the ACCIS database were analysed by five regions: the British Isles, East, North, South, and West. Overall age-standardised incidence rate (ASR) of CNS tumours in Europe (1988–1997) was 29.9 per million, with the highest rates in the North. Astrocytoma (ASR = 11.8), primitive neuroectodermal tumours (PNET) (ASR = 6.5) and ependymoma (ASR = 3.4) were the most frequent types. Incidence increased significantly during 1978–1997, on average by 1.7% per year. Diagnostic methods may partially explain incidence rates and trends, although a role of variations in risk factors cannot be excluded. Overall 5-year survival was 64% and varied between 72% in the North and 53% in the East. PNET had the poorest prognosis (49%) and astrocytoma the best (75%). Survival has improved by 29% since late 1970s. The positive trends were seen in all regions, although the interregional differences persisted, as a reflection of the different healthcare systems.

Introduction

Neoplasms of the central nervous system (CNS) represent 20% of all childhood cancer (age 0–14 years). They are the second most common group of neoplasms (after leukaemias) and the largest group of solid tumours in children of developed countries. In the 1980s and early 1990s, the incidence rates of CNS tumours ranged between 20 and 40 per million in Europe and North America.1 Lower rates were observed in developing countries, which might reflect ethnic variations, differences in socio-economic factors,2, 3 but also lower availability of non-invasive diagnostic techniques.4 CNS tumours are the second cause of death from childhood cancer in Europe5 and North America.6 Survival in developed countries improved markedly,6, 7, 8 but many survivors of CNS tumours suffer considerable sequelae that may imply lifetime medical surveillance.9

CNS tumours are a heterogeneous collection of neoplasms of different histology, behaviour and prognosis. Paediatric CNS tumours differ from adult CNS tumours considerably by histology and anatomical site. Main diagnostic groups in children are astrocytoma (38–50%), ependymoma (8–14%), primitive neuroectodermal tumours (PNET), including medulloblastoma (16–25%), and other gliomas (4–16%).1 There are large variations among these groups of childhood CNS tumours in terms of prognosis and response to therapies even within diagnostic categories. For example, ‘astrocytoma’ includes a variety of tumours, ranging from slowly growing pilocytic astrocytoma with a very good prognosis, to extremely malignant glioblastoma multiforme with some 2% 3-year survival.10 The proportion of various histologies in a group may thus bias the interpretation of survival results. Vulnerability of the brain to therapies, especially in young ages, is a challenge for clinical management.11

The aim of this paper is to describe the incidence and survival of childhood CNS tumours in Europe and their geographical and temporal variations using a large European database of the Automated Childhood Cancer Information System (ACCIS).12

Section snippets

Material and methods

All 19,531 malignant and non-malignant tumours of the central nervous system (CNS), registered between 1978 and 1997 in patients aged less than 15 years and resident in the geographical areas covered by the participating registries at the time of diagnosis were extracted from the ACCIS database. Given a satisfactory evaluation of data comparability by the ACCIS Scientific Committee [Steliarova-Foucher, Kaatsch, Lacour and colleagues, this issue], 59 cancer registries in 19 European countries

Results

During 1988–1997, 11,829 cases of a primary CNS tumour were recorded in the participating registries (Table 3). Globally, 88% of cases were microscopically verified, ranging from 78% (British Isles) to 95% (West). In Europe, less than 3% of cases were diagnosed from death certificate only (in the set of registries with access to this source of data) or from an unknown source. Largest inter-regional variations were observed in the unspecified group, followed by the other gliomas group. In these

Discussion

The present study is the largest report on incidence and survival of children with CNS tumours in Europe. The results are based on comparable data from 59 population-based cancer registries with high standards of main indicators of data quality. Despite rigorous data verification and careful evaluation of the comparability of the data-sets, it was not possible to account for all differences in diagnostic, registration and coding practices, specific to CNS tumours, which may have affected the

Conflict of interest statement

None declared.

Acknowledgements

The ACCIS project was funded by the European Commission from ‘Europe Against Cancer’ action program (1996–2002)_(contracts SI2.126875, SI2.321970 and SPC.2002303), jointly with the International Agency for Research on Cancer (IARC). Data analyses were partly financed by the French Ligue Nationale Contre le Cancer, Comité du Rhône. In the RNTI-SEOP this study was partially supported by Fundación Científica AECC, ISCIII Network RITSI G03/089, Fundación Villavecchia and Fundación SEOP. In Granada

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