Automated Childhood Cancer Information System Research Articles

Childhood and adolescent lymphoma in Spain: incidence and survival trends over 20years.

Lymphoma is the third most common malignancy in children (0-14years) and the first in adolescents (15-19years). This population-based study-the largest ever done in Spain-analyses incidence and survival of lymphomas among Spanish children and adolescents. 1664 lymphoma cases (1983-2007) for incidence and 1030 for survival (1991-2005) followed until 31/12/2010, were provided by 11 cancer registries. Age-adjusted incidence rates (ASRw) to the world standard population were obtained; incidence trends were modelled using the Joinpoint programme, observed survival (OS) was estimated with Kaplan-Meier and trends tested with a log-rank test. Results are presented according to the International Classification of Childhood Cancer-3. In Spain, the ASRw0-14 for lymphomas was 17.5 per 1.000.000 child-years and 50.0 the specific rate for adolescents. Overall incidence increased significantly during 1983-1997 with no increases thereafter. Patients over 9years old showed significant rising trends for all subtypes, except for Burkitt lymphoma (BL) in adolescents. During 2001-2005 (age 0-19years), 5-year OS was 94 (90-98), 73 (64-83) and 86 (78-94) for Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL) and BL, respectively. No improvement in survival was found. The incidence in Spain was higher than overall European rates, but within the range of that in Southern Europe. Comparing OS in Spain 1991-1995 and 2001-2005 with results for Europe of the Automated Childhood Cancer Information System (ACCIS) (1988-1997) and the European cancer registry-based study on survival and care of cancer patients (EUROCARE) (2000-2007), it was similar for HL and lower for NHL and BL. Systematic monitoring and analysis of lymphoma paediatric data would provide clinical and epidemiological information to improve the health care of these patients and the outcomes for these malignancies in Spain.

Incidence, time trends and regional variation of childhood leukaemia in Germany and Europe

This paper presents data on the German and Europe-wide incidence, time trends and regional variations of childhood leukaemia. Data were provided by the German Childhood Cancer Registry (GCCR), a population-based cancer registry recording all cases of malignant diseases in children under 15 y of age residing in Germany and by the Automated Childhood Cancer Information System (ACCIS) co-ordinated at International Agency for Research on Cancer, Lyon, that combines and evaluates data from several European population-based cancer registries. The incidence of leukaemia (44.0 per million) has increased in Europe as well as in Germany in the last decades (0.6% annually on average). Germany shows no systematic aggregation of regions with low or high cancer incidence in terms of regional clustering. Incidence rates differ between European regions with the highest rates in Northern Europe (48.0 per million) and the lowest rates in Eastern Europe (39.1). Altogether, the results from ACCIS and the GCCR show good agreement.

Childhood leukaemias and lymphomas in Greece (1996–2006): a nationwide registration study

Background: Monitoring time trends in the incidence of childhood leukaemias and lymphomas requires efficient and continuous data collecting systems. In countries without official cancer registries, such as Greece, ad hoc...

Poor accrual of teenagers and young adults into clinical trials in the UK

There is now a greater recognition within the international cancer community that teenagers and young adults, broadly those aged between 13 and 24 years, deserve specific attention for a set of unique needs. Alongside the challenges set by a cancer diagnosis during a period of intense personal change and development, data from Europe and the USA have shown that improvements in survival for teenagers and young adults have lagged behind those of their paediatric and adult counterparts. 1 Bleyer A Montello M Budd T Saxman S National survival trends of young adults with sarcoma: lack of progress is associated with lack of clinical trial participation. Cancer. 2005; 103: 1891-1897 Crossref PubMed Scopus (134) Google Scholar , 2 Stiller C Desandes E Danon S et al. Cancer incidence and survival in European adolescents (1978–1997). Report from the Automated Childhood Cancer Information System project. Eur J Cancer. 2006; 13: 2006-2018 Summary Full Text Full Text PDF Scopus (83) Google Scholar Why has survival for this age group been unsatisfactory? In seeking explanations, investigators have especially focused on clinical-trial participation. Data from the UK are now emerging, which confirm findings from other parts of the world suggesting that the accrual of teenagers and young adults to trials is much lower than that of younger children with similar cancers. Improving recruitment to clinical trials for cancer in childhoodClinical trials have underpinned progress in the treatment of cancer in childhood: about 75% of children newly diagnosed with cancer can expect to be long-term survivors. This success has been achieved because most children with cancer have participated in available clinical trials. This high level of engagement of the childhood-cancer community relies on the so-called therapeutic alliance that begins between doctors and families when a child is diagnosed with cancer. More research is needed to understand how to present most effectively the unfamiliar idea of a randomised clinical trial at a stressful time. Full-Text PDF

Up-to-date monitoring of childhood cancer long-term survival in Europe: central nervous system tumours

Tumours of the central nervous system (CNS) account for 15-20% of all malignant childhood tumours in developed countries. Steady improvement of survival of children with CNS tumours has been reported for the past decades. However, these results, obtained by cohort analysis of survival, do not reflect the full extent of recent improvement. Using selected registries from the database of the Automated Childhood Cancer Information System (ACCIS), we calculated period survival estimates for the years 1995-99 for children diagnosed with a malignant CNS tumour. The overall 10-year period survival estimate for the years 1995-99 was 59% for children with all CNS tumours combined, 73% for children with astrocytoma, 53% for children with ependymoma and 45% for children with primitive neuroectodermal tumours. On average, estimates derived by cohort analysis (pertaining to children diagnosed in 1985-89) were around 4% units lower. Region-specific analysis revealed that recent progress was largest in Eastern Europe, where prognosis nevertheless remained lower than in other European regions. In Northern and Southern Europe, 10-year survival remained essentially unchanged. Although period survival of children with CNS tumours is higher than previously reported cohort survival, their long-term prognosis remains modest compared to other childhood malignancies.

Up-to-date monitoring of childhood cancer long-term survival in Europe: tumours of the sympathetic nervous system, retinoblastoma, renal and bone tumours, and soft tissue sarcomas

Prognosis for most types of childhood tumours has improved during the last few decades. In this article we estimate up-to-date period survival for less common, but important childhood malignancies in Europe. Using the database of the Automated Childhood Cancer Information System we calculated period estimates of 10-year survival for the 1995-1999 period for children aged 0-14 years diagnosed during 1985-1999 with tumours of the sympathetic nervous system (NS), retinoblastoma, renal tumours, bone tumours and soft tissue sarcomas in four European regions. Ten-year period survival for 1995-1999 was 66% in children with tumours of the sympathetic NS, 96% for retinoblastoma, 87% for renal tumours, 58% for bone tumours and 61% for soft tissue sarcomas. The higher period estimates, as compared with cohort and complete estimates indicate recent improvement in survival for tumours of the sympathetic NS and to a lesser extent for retinoblastoma and renal tumours. Region-specific period survival estimates were lowest for Eastern Europe for renal, bone and soft tissue tumours, but not for the other two tumour groups. There have been further improvements in the 1990s in long-term survival of children diagnosed with several malignancies, albeit to a different extent in different European regions.

Timely disclosure of progress in childhood cancer survival by ‘period’ analysis in the Automated Childhood Cancer Information System

A few years ago, a new method of survival analysis, denoted 'period' analysis, was introduced to provide more up-to-date survival estimates of cancer patients. We evaluated the period survival method using the large database of the Automated Childhood Cancer Information System (ACCIS). Our evaluation is based on data from 35 191 children diagnosed with cancer in 13 European countries between 1975 and 1989 and followed for vital status until around 1999. Using the follow-up data available in 1989, 10-year survival for all children with cancer calculated by the period method for the 1985-89 period was 58%, while it was 43% when calculated by traditional 'cohort' life-table analysis (based on children diagnosed in 1975-79). The period method provided a better estimate of the true 10-year survival of 62%, observed 10 years later in the cohort of patients diagnosed in 1985-89. Similar results were observed for each of the common groups of childhood cancer. Period analysis is especially useful for monitoring childhood cancer survival, because at a given point in time it provides more timely estimates of long-term survival expectations than the cohort life-table method. Using the ACCIS database, up-to-date estimates of period survival for childhood cancer are derived in subsequent papers in this journal.

Up-to-date monitoring of childhood cancer long-term survival in Europe: methodology and application to all forms of cancer combined

The Automated Childhood Cancer Information System (ACCIS) provides a unique database for monitoring and comparing long-term prognosis of children with cancer in Europe. The 'period' method has been shown particularly useful for that purpose. The ACCIS database was used for comparative analyses of up-to-date long-term survival in four regions and 19 countries of Europe by period analysis. Overall, follow-up data on 49,503 children diagnosed between 1985 and the end of the 1990s were included. Period estimates of 10-year survival were derived for the 1995-99 period and compared with estimates obtained by cohort and complete analysis. In this article, the methodology is described and the approach is illustrated for all forms of childhood cancer combined. Long-term childhood cancer survival achieved in Europe by the end of the twentieth century is much higher than previous estimates indicated. The overall period estimate of 10-year survival was 71% for all countries combined, but it varied between 45% for Estonian children and 79% for Swedish and Finnish children with cancer. Period analysis is the method of choice to monitor population-based survival. Despite major improvement during the past decades, tremendous variation in childhood cancer survival between European countries has persisted.

Geographical comparison of cancer survival in European children (1988–1997): Report from the Automated Childhood Cancer Information System project

The aim of this study was to assess regional survival differences among childhood cancer patients in Europe. For this exercise, the Automated Childhood Cancer Information System (ACCIS) database was utilised. Survival data from 54 population-based cancer registries on 49,651 childhood cancer patients aged 0–14 years and diagnosed in 1988–1997 were analysed using life-table method. Overall, the 5-year survival was 72% among all patients, varying from 62% to 77% between the five geographical regions. The East region generally had lower survival rates than the rest of Europe. The geographical differences indicate the need for more co-ordination, systematisation and standardisation in diagnosis, referral and the treatment of childhood cancers in Europe. Increase of resources is necessary to improve the lower survival in the East region. Continuing data collection on a European level will facilitate monitoring of population-based survival of childhood cancer patients.

Time trends of cancer incidence in European children (1978–1997): Report from the Automated Childhood Cancer Information System project

Within the framework of the Automated Childhood Cancer Information System (ACCIS), time trend analyses for childhood cancer were performed using data from 33 population-based cancer registries in 15 European countries for the period 1978–1997. The overall incidence rate based on 77,111 cases has increased significantly ( P < 0.0001), with an average annual percentage change (AAPC) of 1.1%. The rising trend was observed in all five geographical regions and in the majority of the disease groups (in order of AAPC): soft tissue sarcomas (1.8%), brain tumours, tumours of the sympathetic nervous system, germ-cell tumours, carcinomas, lymphomas, renal tumours, and leukaemias (0.6%). No change was seen in incidence of bone tumours, hepatic tumours and retinoblastoma. The increased incidence can only partly be explained by changes in diagnostic methods and by registration artefacts. The patterns and magnitude of these increases suggest that other factors, e.g. changes in lifestyle and in exposure to a variety of agents, have contributed to the increase in childhood cancer in the recent decades.

Thyroid cancer incidence and survival among European children and adolescents (1978–1997): Report from the Automated Childhood Cancer Information System project

Data on 1690 childhood and adolescent cases of thyroid cancer registered in 61 European cancer registries were extracted from the database of the Automated Childhood Cancer Information System (ACCIS) and included in analyses of incidence and survival. In 1988–1997, the age-standardised incidence rates (ASR) for children aged 0–14 years varied in European regions from 0.5 to 1.2 per million and the age-specific incidence in adolescents aged 15–19 years ranged from 4.4 to 11.0 per million. Over the age-span 0–19 years, the female to male ratio increased from 1 to around 3. Papillary thyroid cancer accounted for almost 65% of cases in children and 77% in adolescents. In the childhood population of Belarus, the ASR for 1989–1997 was 23.6 per million and the proportion of papillary tumours was 87%. No association was found between thyroid cancer risk and national dietary iodine status across 16 countries. Incidence of thyroid carcinoma among children and adolescents in Europe (excluding Belarus) increased during 1978–1997 by 3% per year, largely due to papillary carcinoma. Survival of children and adolescents was high over the entire study period and in all regions of Europe. Children with medullary carcinoma had slightly lower 5-year survival (95%, 95% CI 81–99), than those with papillary carcinoma (99%, 95% CI 95–100). More than 90% of patients survived 20 years after diagnosis. Further standardisation of diagnostic, classification and registration criteria will be fundamental for future studies of thyroid carcinomas in young people.

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

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.

Bone tumours in European children and adolescents, 1978–1997. Report from the Automated Childhood Cancer Information System project

Data on 5572 children and adolescents diagnosed with malignant bone tumours (International Classification of Childhood Cancer, Group VIII) before the age of 20 years during 1978–1997 in Europe were extracted from the Automated Childhood Cancer Information System (ACCIS) database. Age-standardised incidence among children during the period 1988–1997 was similar for boys and girls aged 0–14 years (5.5–5.6 per million). Among adolescents aged 15–19 years, males had higher incidence (19.3 per million) than females (10.7 per million). Among children, osteosarcoma accounted for 51% of registrations and Ewing’s sarcoma for 41%. Among adolescents, 55% of registrations were osteosarcoma and 28% Ewing’s sarcoma. Both tumours had their highest incidence in late childhood or early adolescence. There were no significant time trends in incidence during 1978–1997. Five-year survival estimates for patients diagnosed during 1988–1997 were, respectively, 59% and 51% among children and adolescents with osteosarcoma and 62% and 30% among children and adolescents with Ewing’s sarcoma. Between 1978–1982 and 1993–1997, survival increased for both children and adolescents with osteosarcoma, and for children with Ewing’s sarcoma.

Retinoblastoma incidence and survival in European children (1978–1997). Report from the Automated Childhood Cancer Information System project

Based on 2283 cases of retinoblastoma diagnosed in children aged 0–14 years, incidence and survival in Europe during the period 1978–1997 are described. Data were provided to the Automated Childhood Cancer Information System (ACCIS) from 60 paediatric and general cancer registries. During 1988–1997, the cumulative incidence of retinoblastoma in the ACCIS regions was found to be between 44.2 and 67.9 per million births. The highest incidence was seen in the first year of life. The age-standardised (World standard) incidence rate for the age-range 0–14 years was 4.1 per million. Approximately one-third of cases had bilateral tumours. Overall incidence increased over the period 1978–1997 by 1% per year, as derived from a model adjusted for sex, age group and type of registry (general or paediatric). The 5-year survival rate improved from 89% to 95% during the period covered by the study. This improvement was seen in both unilateral and bilateral cases but was significant only for the unilateral tumours. Survival was lower in the East region, although smaller differences were also observed between the other four regions (British Isles, North, South and West). Availability and quality of registration data on retinoblastoma need to be improved for effective evaluation of incidence and survival.

Quality, comparability and methods of analysis of data on childhood cancer in Europe (1978–1997): Report from the Automated Childhood Cancer Information System project

In collaboration with 62 population-based cancer registries contributing to the Automated Childhood Cancer Information System (ACCIS), we built a database to study incidence and survival of children and adolescents with cancer in Europe. We describe the methods and evaluate the quality and internal comparability of the database, by geographical region, period of registration, type of registry and other characteristics. Data on 88,465 childhood and 15,369 adolescent tumours registered during 1978–1997 were available. Geographical differences in incidence are caused partly by differences in definition of eligible cases. The observed increase in incidence rates cannot be explained by biases due to the selection of datasets for analyses, and only partially by the registration of non-malignant or multiple primary tumours. Part of the observed differences in survival between the regions may be due to variable completeness of follow-up, but most is probably explained by resource availability and organisation of care. Further standardisation of data and collection of additional variables are required so that this study may continue to yield valuable results with reliable interpretation.

Cancer incidence and survival in European adolescents (1978–1997). Report from the Automated Childhood Cancer Information System project

Data on 15,399 adolescents diagnosed with cancer at age 15–19 years during 1978–1997 in Europe were extracted from the database of the Automated Childhood Cancer Information System (ACCIS). Total incidence in Europe as a whole was 186 per million in 1988–1997. Incidence among males was 1.2 times that among females. Lymphomas had the highest incidence of any diagnostic group, 46 per million, followed by epithelial tumours, 41 per million; central nervous system (CNS) tumours, 24; germ cell and gonadal tumours, 23; leukaemias, 23; bone tumours, 14; and soft tissue sarcomas, 13 per million. Total incidence varied widely between regions, from 169 per million in the East to 210 per million in the North, but lymphomas were the most frequent diagnostic group in all regions. Cancer incidence among adolescents increased significantly at a rate of 2% per year during 1978–1997. Five-year survival for all cancers combined in 1988–1997 was 73% in Europe as a whole. Survival was highest in the North, 78%, and lowest in the East, 57%. Five-year survival was generally comparable with that in the Surveillance, Epidemiology, and End Results (SEER) registries of the United States of America (USA), but for Ewing’s sarcoma it was below 45% in all European regions compared with 56% in the USA. Survival increased significantly during 1978–1997 for all cancers combined and for all diagnostic groups with sufficient registrations for analysis.

Neuroblastoma incidence and survival in European children (1978–1997): Report from the Automated Childhood Cancer Information System project

The Automated Childhood Cancer Information System (ACCIS) collects and presents data on childhood cancer in Europe. This report describes trends (1978–1997) and geographical differences (1988–1997) in incidence and survival for 6202 children with neuroblastoma from 59 registries in 19 countries, grouped into five regions (British Isles, West, East, North, and South). The age-standardised incidence rate (ASR) of neuroblastoma in Europe in 1988–1997 was 10.9 cases per million children, being highest in infants (52.6). The ASR of neuroblastoma increased in Europe from 8.4 in 1978–1982 to 11.6 in 1993–1997, mostly due to an increase in infants (from 35.4 to 57.8). Overall 5-year survival was 59%, ranging from 47% (East) to 67% (West). It improved markedly from 37% in 1978–1982 to 66% in 1993–1997, especially in infants. A certain amount of overdiagnosis in children under 2 years of age may explain the increased incidence rates and partially the increase in survival. Survival of older children (aged 2–14 years), which is likely to be largely affected by therapy, has also improved from 21% to 45%.

Childhood soft tissue sarcomas incidence and survival in European children (1978–1997): Report from the Automated Childhood Cancer Information System project

This population-based study is based on 5802 cases of soft tissue sarcomas (STS) in children aged 0–14 years extracted from the database of the Automated Childhood Cancer Information System (ACCIS) and registered in population-based cancer registries in Europe for the period 1978–1997. STS represent almost 8% of neoplasms in children, almost half of whom are less than 5 years at diagnosis. Rhabdomyosarcoma is the most frequent childhood STS (50%). During 1988–1997 the age-standardised incidence of STS in Europe was 9.1 per million children, lowest in the West and East and highest in the North. The incidence of STS increased almost 2% per year over the period 1978–1997, attributable mostly to increase in genito-urinary rhabdomyosarcoma. Prognosis of children with STS was related to age and site of tumour. Five-year survival of children with STS increased from 46% in 1978–1977 to 66% in 1993–1997, reaching 74% in the North for those diagnosed in 1993–1997. This improvement is ascribed to therapy advances.

Skin cancer incidence and survival in European children and adolescents (1978–1997). Report from the Automated Childhood Cancer Information System project

Patterns and trends of melanoma and skin carcinoma incidence and survival of European children (age 0–14 years) and adolescents (age 15–19 years) were investigated. Between 1978 and 1997, a total of 1419 melanoma and 485 skin carcinoma cases were recorded in the cancer registries contributing to the Automated Childhood Cancer Information System (ACCIS) study. During 1988–1997, the incidence of melanoma was 0.7 per million children and 12.9 per million adolescents; corresponding rates for skin carcinomas were 0.3 and 3.7 per million, respectively. The British Isles had the highest incidence of skin cancers in children and adolescents. For Europe, in adolescents melanomas were more common in the North and West, skin carcinomas in the South and East. Between 1978 and 1997 incidence increased annually in adolescents, by 4.1% for melanoma and 2.5% for skin carcinoma. Differences in aetiology between childhood and adolescent skin cancers cannot be excluded. Survival was relatively high and the geographical variations in incidence and survival seem to be associated.

Geographical patterns of childhood cancer incidence in Europe, 1988–1997. Report from the Automated Childhood Cancer Information System project

Data on more than 50,000 registrations in the Automated Childhood Cancer Information System (ACCIS) database were used to present an overview of regional patterns in childhood cancer incidence in Europe during 1988–1997, and to present additional detail on selected carcinomas whose occurrence in childhood is seldom described because of their rarity. Total age-standardised incidence was 138.5 per million for Europe overall, and varied between regions from 131.1 per million in the British Isles to 160.1 per million in Northern Europe. Incidence varied significantly between regions for nearly all diagnostic groups. The greatest range of regional incidence rates was for central nervous system (CNS) tumours, from 27.0 per million in the West to 43.8 per million in the North. Differences in registration practice for non-malignant tumours account for some of this variation. There was a marked excess of carcinoma in Eastern Europe, which was wholly attributable to the high incidence of thyroid carcinoma in Belarus, though there was also evidence of inter-regional variation attributable to differences in registration practice. The geographical heterogeneity of incidence rates for other diagnostic groups seems more likely to reflect variations in underlying risk.