Bone tumours in European children and adolescents, 1978–1997. Report from the Automated Childhood Cancer Information System project
Introduction
Malignant bone tumours account for 3–5% of cancers diagnosed in children under 15 years of age1 and 7–8% of those in adolescents aged 15–19 years in western populations.2 Malignant bone tumours comprise more than 20 different entities, classified according to either the direct product of the tumour cells (e.g. osteoid in osteosarcomas) or the type of tissue they form (e.g. vascular channels in angiosarcoma of bone).3 The great majority of malignant bone tumours occurring in young people under the age of 20 years are osteosarcoma and Ewing’s sarcoma. Most malignant bone tumours, including both of these types, must be regarded as high-grade sarcomas that, at the time of diagnosis, are likely to have spread to distant sites, especially the lungs. In the pre-chemotherapeutic era, their prognosis was very poor, since treatment was focussed on local procedures.
Identified risk factors for osteosarcoma are limited to ionising radiation, e.g. radiotherapy for a previous cancer, and certain genetic conditions including familial retinoblastoma, Li-Fraumeni syndrome and Rothmund-Thomson syndrome.4, 5 Ewing’s sarcoma has long been known to have lower incidence among black and East Asian populations,6 indicating that genetic factors may be important in its aetiology. Cytogenetic differences between Ewing’s tumours from European and Japanese patients lend further support to this suggestion.7 Analyses of combined data from several case-control studies in North America and Australia have confirmed earlier findings of a raised risk with a history of umbilical hernia, possibly with a common environmental cause,8 and with parental occupation in farming, though with no indication of the relative importance of exposure to particular categories of chemicals or animals.9
This paper presents geographical patterns and time trends in the incidence and survival rates for malignant bone tumours among children and adolescents in Europe and identifies needs for further studies. The analyses are based on a large European database within the Automated Childhood Cancer Information System (ACCIS), which contains data from 80 population-based cancer registries in 35 participating countries.10
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Material and methods
Malignant bone tumours were defined as all those neoplasms in group VIII of the International Classification of Childhood Cancer (ICCC).11 All 5572 malignant bone tumours registered since 1978 in patients under 20 years of age in 59 participating cancer registries of 19 European countries were extracted from the ACCIS database (Table 1). This number included 2883 osteosarcomas (ICCC subgroup VIIIa), 231 chondrosarcomas (ICCC subgroup VIIIb), 2130 Ewing’s sarcomas (ICCC subgroup VIIIc), 165
Results
Table 3 shows incidence of bone tumours during 1988–1997 by 5-year age group among males and females in Europe as a whole, and Fig. 1 shows incidence by single year of age. Osteosarcoma was the most frequent subgroup, accounting for 52% of all registrations. Ewing’s sarcoma was second most frequent, accounting for 34%. Chondrosarcoma and other specified tumours accounted for 6% and 4%, respectively, and 4% of registrations were for tumours of unspecified type. All types of bone tumour were very
Discussion
This is the largest ever population-based study of bone tumours in children and adolescents. There was some evidence of geographical variation in incidence within Europe, but no significant change in rates over time. Survival rates were generally higher for children than for adolescents, and were markedly lower in the East than in the other European regions. Survival increased substantially during the 20-year study period.
The total incidence of malignant bone tumours in childhood, the
Conflict of interest statement
None declared.
Acknowledgements
The ACCIS project was funded by the European Commission from the ’Europe Against Cancer’ programme (1996–2002, contracts SI2.126875, SI2.321970 and SPC.2002303), jointly with the International Agency for Research on Cancer (IARC). Data analyses were partly funded by the French Ligue Nationale contre le Cancer, Comité du Rhône. The Childhood Cancer Research Group receives funding from the Department of Health and the Scottish Ministers. The views expressed in the publication are those of the
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