Childhood soft tissue sarcomas incidence and survival in European children (1978–1997): Report from the Automated Childhood Cancer Information System project
Introduction
Soft tissue sarcomas (STS) are a heterogeneous group of neoplasms developing from mesenchymal cell at any site of the body. STS comprise about 8% of all childhood cancers. Almost half of the patients with STS are less than 5 years of age at diagnosis. These neoplasms are categorised in two broad groups: rhabdomyosarcoma (RMS) and non-rhabdomyosarcoma STS (NRSTS). RMS is the single most frequent childhood STS, accounting for more than 50% of cases, and fibrosarcoma is the most common NRSTS, accounting for 10–20% of STS cases. The primary site is related to age at diagnosis and histological subtype.1
STS rarely occurs as part of congenital malformation syndromes. Patients with Li-Fraumeni syndrome, neurofibromatosis type 1 and other rare familial syndromes are at increased risk of developing STS. However, these genetic susceptibility conditions explain only a small proportion of childhood cases.2, 3 Characteristic somatic changes are associated with specific types of STS.4 Radiation therapy is the best-known environmental risk factor for STS.2, 5 HIV-infected patients are at a high risk of developing a specific STS, Kaposi’s sarcoma (KS). KS is a complex multifactorial virally driven process: the HIV infection is necessary, but not sufficient for its development. However, the risk is lower among HIV-infected children in Europe and in North America than in Africa: this pattern is presumably related to a different prevalence of cofactors.6
Previous EUROCARE studies evaluated population-based survival of children diagnosed with STS in European Countries before 1994.8, 11 The prognosis of RMS is related to age, tumour site and size, histology and extent of disease at diagnosis. The findings of a large clinical series7 are consistent with the results of the population-based study EUROCARE-28 and suggest poor prognosis for infants and adolescents with RMS, compared with children aged 1–9 years.
Over the last 40 years, the prognosis of children with RMS has improved from 25% in 1970s to 70% in 1990s due to use of multimodal therapy, as well as to development of an armamentarium of effective cytotoxic drugs.4, 7 The improvement in treatment strategies for RMS illustrates the value of multi-institutional co-operative groups to study large series of rare childhood tumours with clinical and biological heterogeneity.7
Until recently, the clinical relevance of histological classification of NRSTS was limited. Most of the clinical knowledge and experience gathered in the management of children with RMS has been applied also in the treatment of children with NRSTS, even though these are different biological entities with variable chemosensitivity.4, 9, 10
The aim of this report is to describe incidence and survival of STS in children in Europe and their geographical and temporal variations and suggest the direction for further studies. The present study benefits from a large population-based series of data, collected in the framework of the Automated Childhood Cancer Information System (ACCIS) from 80 European cancer registries for the period 1978–1997.12
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Material and methods
This study is based on the data from 59 cancer registries in 19 European countries (Table 1) retrieved from the ACCIS database, and evaluated as comparable by the ACCIS Scientific Committee [Steliarova-Foucher, Kaatsch, Lacour and colleagues, this issue]. All malignant STS, as defined by the group IX of the International Classification of Childhood Cancer (ICCC), were included in the analyses.13
In total 5802 cases of STS in children, aged 0–14 years of age, living in the geographical areas
Incidence
For the period 1988–1997 there were 3571 cases of newly diagnosed STS in children younger than 15 years of age included in the analysis (Table 4). They represented 6.6% of all childhood cancers in this data-set [Stiller, Marcos-Gragera, Ardanaz and colleagues, this issue]. The diagnosis of great majority of cases (99%) was microscopically verified (MV), and very few were based on death certificate only (DCO) (0.3%) in the registries with access to such information. There were 6% of cases
Discussion
The present study is the largest report on incidence and survival of childhood STS. The ACCIS database provided a uniquely large data-set of almost 6000 cases. The analyses were performed including only quality registries considered comparable. This does not preclude remaining differences in the diagnostic, registration and coding practices. Main indicators of data quality (proportions of microscopically verified cases, cases registered from death certificate only and those classified as
Conflict of interest statement
None declared.
Acknowledgement
The ACCIS project was funded by the European Commission ffrom the Europe Against Cancer programme (contracts SI2.126875, SI2.321970 and SPC.2002303), jointly with International Agency for Research on Cancer. Data analyses were partly funded by the French Ligue Nationale Contre le Cancer, Comité du Rhône. The activity of the Childhood Cancer Registry of Piedmont (CCRP) is supported by the Piemonte Region and by the Italian Association for Research on Cancer. In the RNTI-SEOP this work was
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