Non-Hodgkin’s lymphoma incidence and survival in European children and adolescents (1978–1997): Report from the Automated Childhood Cancer Information System project
Introduction
Non-Hodgkin’s lymphomas (NHLs) constitute a heterogeneous group of malignant tumours, representing 6% of cancers both in children and in adolescents in populations of European descent. 1, 2 Mature B-cell neoplasia comprise over 85% of NHL worldwide, of these only Burkitt’s lymphoma (BL) and large B-cell lymphomas occur with any significant frequency in children.3
Classification of lymphomas, based on morphological features is not simple,4 as documented by the constant search for best classification.5, 6, 7 The increasingly widespread use of molecular and genetic markers in diagnostics is likely to influence further the classification,8 prognostic stratification and tailored therapeutic strategies for lymphomas. The aetiology of these tumours is largely unknown. The major known risk factor for mature B-cell neoplasia appears to be an abnormality of the immune system, notably infection with the human immunodeficiency virus (HIV),3 which is partly responsible for the high rates of some forms of lymphomas in countries heavily affected by HIV, such as Uganda.2 Another infectious agent is the Epstein Barr virus (EBV), found in about 30% of cases of the endemic variant of Burkitt’s lymphoma in industrialised countries.9 Genetic pathways cannot be excluded, notably translocation of Myc in cases of BL, although this is not entirely specific and has been reported in other tumours.10
The project Automated Childhood Cancer Information System (ACCIS) constitutes a large database of childhood and adolescent cancer cases registered in some 80 European regions since 1970.11 The aim of this study is to describe in detail geographical and temporal pattern of incidence of NHL and survival of affected children and adolescents in Europe and interpret the observed results.
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Materials and methods
All lymphomas that were not classified as Hodgkin’s lymphoma were included in this study, namely the following diagnostic subgroups of the International Classification of Childhood Cancer (ICCC):12 Non-Hodgkin’s lymphoma (IIb), Burkitt’s lymphoma (IIc), Miscellaneous lymphoreticular neoplasms (IId) and Unspecified lymphomas (IIe). The remaining lymphoma subgroup, Hodgkin’s disease (IIa) is the subject of another study [Clavel and colleagues, this issue]. The classification rules are reprinted
Results
For the decade 1988–1997, 3850 children and 708 adolescents were included in the analyses (Table 3). The combined group of NHL constituted a variable proportion of neoplasm in children and adolescents: 1.7% in infants, 4.5% in children aged 1–4 years, 10% in age group 5–9 years, 9.6% in age-group 10–14 years and 8% in adolescents. Overall proportion in the age-range 0–14 years was 7.2%. Male to female ratio was 2.4, varying between 1 and 3 over the age units. The proportion of microscopically
Discussion
Incidence and survival data presented here are based on the ACCIS database, resulting from collaboration of European population-based cancer registries. A detailed review of each registry-specific dataset aims at ensuring highest possible quality and comparability [Steliarova-Foucher, Kaatsch, Lacour and colleagues, this issue]. Due to strict criteria, 2124 NHL cases contained in the ACCIS database were excluded from this study.
The high proportion of cases with microscopic verification observed
Conflict of interest statement
None declared.
Acknowledgement
The authors thank Mr Nicolas Mitton for his input in the set-up and management and exploration of the ACCIS database, the members of ACCIS Scientific Committee for steering the study, the Guest Editors for comments on earlier drafts and Ms M.J. Esteban for her secretarial and graphics assistance.
The following collaborators from the cancer registries contributed actively to this study: S.V. Petrovich, O. Budanov (Belarus); H. Storm, N. Christensen (Denmark); T. Aareleid (Estonia); T. Hakulinen,
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