Elsevier

European Journal of Cancer

Volume 42, Issue 13, September 2006, Pages 2050-2063
European Journal of Cancer

Non-Hodgkin’s lymphoma incidence and survival in European children and adolescents (1978–1997): Report from the Automated Childhood Cancer Information System project

https://doi.org/10.1016/j.ejca.2006.05.005Get rights and content

Abstract

Non-Hodgkin’s lymphomas (NHLs) constitute a large and heterogeneous group of malignant tumours. This paper describes and interprets geographical patterns (1988–1997) and time trends (1978–1997) of NHL incidence and survival in European children and adolescents. All 7702 lymphomas that were not Hodgkin’s, were extracted from the Automated Childhood Cancer Information System (ACCIS) database and included in different analyses. In children under 15 years of age and for the period 1988–1997, the overall NHL age-adjusted incidence rate was 9.4 per million and has been increasing over 20 years by 0.9% per year on average (P = 0.002). In adolescents aged 15–19 years, the age-specific incidence rate was 15.9 per million, increasing annually by 1.7% (P = 0.007). Five-year survival of children diagnosed in 1988–1997 was 77%, ranging from 58% in the East to 83% in the West. A substantial increase in survival was observed in all European regions. Systematic monitoring and evaluation of childhood and adolescent data on NHL will contribute to further improvement in public health policy for the young population of Europe.

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.

Section snippets

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,

References (33)

  • Non-Hodgkin’s lymphoma pathologic classification project. National Cancer Institute sponsored study of classification...
  • A.G. Stanfeld et al.

    Updated Kiel classification for lymphomas

    Lancet

    (1988)
  • G. Gaidano et al.

    Lymphomas

  • N.E. Mueller et al.

    Viruses

  • J. Diebold et al.

    Burkitt lymphoma

  • E. Kramárová et al.

    The International Classification of Childhood Cancer

    Int J Cancer

    (1996)
  • Cited by (0)

    View full text