Search

BACKGROUND Leukaemias comprise a heterogenous group of haematological malignancies. In CONCORD-3, we analysed data for children (aged 0-14 years) and adults (aged 15-99 years) diagnosed with a haematological malignancy during 2000-14 in 61 countries. Here, we aimed to examine worldwide trends in survival from leukaemia, by age and morphology, in young patients (aged 0-24 years). METHODS We analysed data from 258 population-based cancer registries in 61 countries participating in CONCORD-3 that submitted data on patients diagnosed with leukaemia. We grouped patients by age as children (0-14 years), adolescents (15-19 years), and young adults (20-24 years). We categorised leukaemia subtypes according to the International Classification of Childhood Cancer (ICCC-3), updated with International Classification of Diseases for Oncology, third edition (ICD-O-3) codes. We estimated 5-year net survival by age and morphology, with 95% CIs, using the non-parametric Pohar-Perme estimator. To control for background mortality, we used life tables by country or region, single year of age, single calendar year and sex, and, where possible, by race or ethnicity. All-age survival estimates were standardised to the marginal distribution of young people with leukaemia included in the analysis. FINDINGS 164 563 young people were included in this analysis: 121 328 (73·7%) children, 22 963 (14·0%) adolescents, and 20 272 (12·3%) young adults. In 2010-14, the most common subtypes were lymphoid leukaemia (28 205 [68·2%] patients) and acute myeloid leukaemia (7863 [19·0%] patients). Age-standardised 5-year net survival in children, adolescents, and young adults for all leukaemias combined during 2010-14 varied widely, ranging from 46% in Mexico to more than 85% in Canada, Cyprus, Belgium, Denmark, Finland, and Australia. Individuals with lymphoid leukaemia had better age-standardised survival (from 43% in Ecuador to ≥80% in parts of Europe, North America, Oceania, and Asia) than those with acute myeloid leukaemia (from 32% in Peru to ≥70% in most high-income countries in Europe, North America, and Oceania). Throughout 2000-14, survival from all leukaemias combined remained consistently higher for children than adolescents and young adults, and minimal improvement was seen for adolescents and young adults in most countries. INTERPRETATION This study offers the first worldwide picture of population-based survival from leukaemia in children, adolescents, and young adults. Adolescents and young adults diagnosed with leukaemia continue to have lower survival than children. Trends in survival from leukaemia for adolescents and young adults are important indicators of the quality of cancer management in this age group. FUNDING Children with Cancer UK, the Institut National du Cancer, La Ligue Contre le Cancer, Centers for Disease Control and Prevention, Swiss Re, Swiss Cancer Research foundation, Swiss Cancer League, Rossy Family Foundation, US National Cancer Institute, and the American Cancer Society.

Background: The EUROCARE-5 study revealed disparities in childhood cancer survival among European countries, giving rise to important initiatives across Europe to reduce the gap. Extending its representativeness through increased coverage of eastern European countries, the EUROCARE-6 study aimed to update survival progress across countries and years of diagnosis and provide new analytical perspectives on estimates of long-term survival and the cured fraction of patients with childhood cancer. Methods: In this population-based study, we analysed 135 847 children (aged 0–14 years) diagnosed during 2000–13 and followed up to the end of 2014, recruited from 80 population-based cancer registries in 31 European countries. We calculated age-adjusted 5-year survival differences by country and over time using period analysis, for all cancers combined and for major cancer types. We applied a variant of standard mixture cure models for survival data to estimate the cure fraction of patients by childhood cancer and to estimate projected 15-year survival. Findings: 5-year survival for all childhood cancer combined in Europe in 2010–14 was 81% (95% CI 81–82), showing an increase of three percentage points compared with 2004–06. Significant progress over time was observed for almost all cancers. Survival remained stable for osteosarcomas, Ewing sarcoma, Burkitt lymphoma, non-Hodgkin lymphomas, and rhabdomyoscarcomas. For all cancers combined, inequalities still persisted among European countries (with age-adjusted 5-year survival ranging from 71% [95% CI 60–79] to 87% [77–93]). The 15-year survival projection for all patients with childhood cancer diagnosed in 2010–13 was 78%. We estimated the yearly long-term mortality rate due to causes other than the diagnosed cancer to be around 2 per 1000 patients for all childhood cancer combined, but to approach zero for retinoblastoma. The cure fraction for patients with childhood cancer increased over time from 74% (95% CI 73–75) in 1998–2001 to 80% (79–81) in 2010–13. In the latter cohort, the cure fraction rate ranged from 99% (95% CI 74–100) for retinoblastoma to 60% (58–63) for CNS tumours and reached 90% (95% CI 87–93) for lymphoid leukaemia and 70% (67–73) for acute myeloid leukaemia. Interpretation: Childhood cancer survival is increasing over time in Europe but there are still some differences among countries. Regular monitoring of childhood cancer survival and estimation of the cure fraction through population-based registry data are crucial for evaluating advances in paediatric cancer care. Funding: European Commission.

Linkage between clinical databases and population-based cancer registries may serve to evaluate European Reference Networks' (ERNs) activity, by monitoring the proportion of patients benefiting from these and their impact on survival at a population level. To test this, a study targeting neuroblastoma (Nb) was conducted in Spain by the European Joint Action on Rare Cancers.Subjects: Nb cases, incident 1999-2017, aged 15 years. Linkage included: Spanish Neuroblastoma Clinical Database (NbCDB) (1217 cases); Spanish Registry of Childhood Tumours (RETI) (1514 cases); and 10 regional population-based registries (RPBCRs) which cover 33% of the childhood population (332 cases). Linkage was semiautomatic. We estimated completeness, incidence, contribution, deficit, and 5-year survival in the databases and specific subsets.National completeness estimates for RETI and NbCDB were 91% and 72% respectively, using the Spanish RPBCRs on International Incidence of Childhood Cancer (https://iicc.iarc.fr/) as reference. RPBCRs' specific contribution was 1.6%. Linkage required manual crossover in 54% of the semiautomatic matches. Five-year survival was 74% (0-14 years) and 90% (0-18 months).All three databases were incomplete as regards Spain as a whole and should therefore be combined to achieve full childhood cancer registration. A unique personal patient identifier could facilitate such linkage. Most children have access to Nb clinical trials. Consolidated interconnections between the national registry and clinical registries (including ERNs and paediatric oncology clinical groups) should be established to evaluate outcomes.

We have analysed incidence and survival trends of children and adolescents with leukaemia registered in Spanish population-based cancer registries during the period 1983-2007.Childhood and adolescent leukaemia cases were drawn from the 11 Spanish population-based cancer registries. For survival, registries with data for the period 1991-2005 and follow-up until 31-12-2010 were included. Overall incidence trends were evaluated using joinpoint analysis. Observed survival rates were estimated using Kaplan-Meier, and trends were tested using the log-rank test.Based on 2606 cases (2274 children and 332 adolescents), the overall age-adjusted incidence rate (ASRw) of leukaemia was 47.9 cases per million child-years in children and 23.8 in adolescents. The ASRw of leukaemia increased with an annual percentage change of 9.6 % (95 % CI: 2.2-17.6) until 1990 followed by a stabilisation of rates. In adolescents, incidence did not increase. Five-year survival increased from 66 % in 1991-1995 to 76 % in 2001-2005. By age, survival was dramatically lower in infants (0) and adolescents (15-19) than in the other age groups and no improvement was observed. In both children and adolescents, differences in 5-year survival rates among major subgroups of leukaemias were significant.The increasing incidence trends observed in childhood leukaemias during the study period were confined to the beginning of the period. Remarkable improvements in survival have been observed in Spanish children with leukaemias. However, this improvement was not observed in infants and adolescents.

List of ACCIS contributors = Monika Hackl, Anna Zborovskaya, Nadya Dimitrova, Zdravka Valerianova, Ladislav Dušek, Margit Mägi, Alain Monnereau, Jacqueline Clavel, Michel Velten, Anne-Valérie Guizard, Véronique Bouvier, Xavier Troussard, Anne-Sophie Woronoff, Emilie Marrer, Brigitte Trétarre, Marc Colonna, Olivier Ganry, Pascale Grosclaude, Berndt Holleczek, Zsuzsanna Jakab, Laufey Tryggvadóttir, Lucia Mangone, Franco Merletti, Stefano Ferretti, Bianca Caruso, Maria Michiara, Rosario Tumino, Fabio Falcini, Roberto Zanetti, Giovanna Tagliabue, Otto Visser, Giske Ursin, Ryszard Mężyk, Kamila Kepska, José Laranja Pontes, Maja Primic Žakelj, Rafael Fernández-Delgado, Marisa L Vicente Raneda, Enrique Almar Marqués, José Ramón Quirós Garcia, Arantza Lopez de Munain, Rafael Marcos-Gragera, Maria-Jose Sanchez-Perez, Maria Ramos Monserrat, Eva Ardanaz, Jaume Galceran, Staffan Khan, Claudia E Kuehni Christine Bouchardy, Fabio Levi, Isabelle Konzelmann, Sabine Rohrmann Sally Vernon, David H Brewster, Ceri White, Anastasia Dolya Background A deceleration in the increase in cancer incidence in children and adolescents has been reported in several national and regional studies in Europe. Based on a large database representing 1∙3 billion person-years over the period 1991–2010, we provide a consolidated report on cancer incidence trends at ages 0–19 years. Methods We invited all population-based cancer registries operating in European countries to participate in this population-based registry study. We requested a listing of individual records of cancer cases, including sex, age, date of birth, date of cancer diagnosis, tumour sequence number, primary site, morphology, behaviour, and the most valid basis of diagnosis. We also requested population counts in each calendar year by sex and age for the registration area, from official national sources, and specific information about the covered area and registration practices. An eligible registry could become a contributor if it provided quality data for all complete calendar years in the period 1991–2010. Incidence rates and the average annual percentage change with 95% CIs were reported for all cancers and major diagnostic groups, by region and overall, separately for children (age 0–14 years) and adolescents (age 15–19 years). We examined and quantified the stability of the trends with joinpoint analyses. Findings For the years 1991–2010, 53 registries in 19 countries contributed a total of 180335 unique cases. We excluded 15162 (8·4%) of 180335 cases due to differing practices of registration, and considered the quality indicators for the 165173 cases included to be satisfactory. The average annual age-standardised incidence was 137∙5 (95% CI 136∙7–138∙3) per million person-years and incidence increased significantly by 0∙54% (0∙44–0∙65) per year in children (age 0–14 years) with no change in trend. In adolescents, the combined European incidence was 176∙2 (174∙4–178∙0) per million person-years based on all 35138 eligible cases and increased significantly by 0·96% (0∙73–1∙19) per year, although recent changes in rates among adolescents suggest a deceleration in this increasing trend. We observed temporal variations in trends by age group, geographical region, and diagnostic group. The combined age-standardised incidence of leukaemia based on 48458 cases in children was 46∙9 (46∙5–47∙3) per million person-years and increased significantly by 0∙66% (0∙48–0∙84) per year. The average overall incidence of leukaemia in adolescents was 23∙6 (22∙9–24∙3) per million person-years, based on 4702 cases, and the average annual change was 0∙93% (0∙49–1∙37). We also observed increasing incidence of lymphoma in adolescents (average annual change 1·04% [0·65–1·44], malignant CNS tumours in children (average annual change 0·49% [0·20–0·77]), and other tumours in both children (average annual change 0·56 [0·40–0·72]) and adolescents (average annual change 1∙17 [0∙82–1∙53]). Interpretation Improvements in the diagnosis and registration of cancers over time could partly explain the observed increase in incidence, although some changes in underlying putative risk factors cannot be excluded. Cancer incidence trends in this young population require continued monitoring at an international level. Funding Federal Ministry of Health of the Federal German Government, the European Union’s Seventh Framework Programme, and International Agency for Research on Cancer

Cancer is relatively rare in childhood, but it contributes considerably to childhood mortality, years of life lost per person and late effects in survivors. Large populations need to be covered to set up meaningful studies of these rare conditions. Cancer registries ensure cancer surveillance, thus providing the basis for research as well as policy decisions. In this paper we examine coverage of childhood population by cancer registries in Europe and encourage national cancer registration. Over 200 cancer registries in various stages of development were identified as collecting data on childhood cancer patients in Europe. They cover 52% of the childhood population in the World Health Organisation (WHO) European region and 83% in the European Union (EU). More than 80% of this coverage is ensured by nationwide data collection, which is ongoing in 29 European countries. Overall coverage of the childhood population could increase to around 98%, if the recently established cancer registries start producing results and others improve their quality and dissemination plans. Paediatric cancer registries are being established with increasing frequency even in the areas covered by general cancer registries, and they tend to be national. Compared with regional registration, national cancer registries are more cost-effective, record larger number of cases, they can achieve higher completeness, less biased incidence and survival estimates and they are conditioned for national and international research. National registration of childhood cancer should be the rule in Europe, so that accurate regional, nation-wide and international statistics can provide solid baselines for research, clinical practice and public health policy. Governmental support and stakeholders' involvement are indispensable to guarantee optimal data quality and completeness.

Proportion cured is a potentially more informative cancer outcome measurement than 5-year survival. We present population-based estimates of cure for young patients diagnosed with acute lymphoblastic leukemia in Europe from 1982 to 2002. Thirty-five European cancer registries provided data. Survival was estimated by age, period of diagnosis and European region, and used as input for parametric cure models, which assume cured patients have the same mortality as the general population. For acute lymphoblastic leukemia diagnosed in 1–14 year olds in 2000–2002, over 77% were estimated cured. The proportion cured improved significantly over the study period: an impressive 26–58% in infants (up to 1 year), 70–90% in 1–4 year olds, 63–86% in 5–9 year olds, 52–77% in 10–14 year olds, and 44–50% in 15–24 year olds. Regional variations in proportion cured reduced over time for 1–14 year-olds, but persisted in infants and 15–24 year olds. Five-year survival was always slightly higher than proportion cured. Considerable proportions of young patients were estimated cured of acute lymphoblastic leukemia. Nevertheless, a small excess risk of death persisted beyond five years after diagnosis when patients remained at risk for late treatment effects, late relapses and second primaries.

International audience; The present study is aimed to compare survival and prognostic changes over time between elderly (70-84 years) and middle-aged cancer patients (55-69 years). We considered seven cancer sites (stomach, colon, breast, cervix and corpus uteri, ovary and prostate) and all cancers combined (but excluding prostate and non-melanoma skin cancers). Five-year relative survival was estimated for cohorts of patients diagnosed in 1988-1999 in a pool of 51 European populations covered by cancer registries. Furthermore, we applied the period-analysis method to more recent incidence data from 32 cancer registries to provide 1- and 5-year relative survival estimates for the period of follow-up 2000-2002. A significant survival improvement was observed from 1988 to 1999 for all cancers combined and for every cancer site, except cervical cancer. However, survival increased at a slower rate in the elderly, so that the gap between younger and older patients widened, particularly for prostate cancer in men and for all considered cancers except cervical cancer in women. For breast and prostate cancers, the increasing gap was likely attributable to a larger use of, respectively, mammographic screening and PSA test in middle-aged with respect to the elderly. In the period analysis of the most recent data, relative survival was much higher in middle-aged patients than in the elderly. The differences were higher for breast and gynaecological cancers, and for prostate cancer. Most of this age gap was due to a very large difference in survival after the 1st year following the diagnosis. Differences were much smaller for conditional 5-year relative survival among patients who had already survived the first year. The increase of survival in elderly men is encouraging but the lesser improvement in women and, in particular, the widening gap for breast cancer suggest that many barriers still delay access to care and that enhanced prevention and clinical management remain major issues.

In international comparisons of cancer registry based survival it is common practice to restrict the analysis to first primary tumours and exclude multiple cancers. The probability of correctly detecting subsequent cancers depends on the registry's running time, which results in different proportions of excluded patients and may lead to biased comparisons. We evaluated the impact on the age-standardised relative survival estimates of also including multiple primary tumours. Data from 2,919,023 malignant cancers from 69 European cancer registries participating in the EUROCARE-4 collaborative study were used. A total of 183,683 multiple primary tumours were found, with an overall proportion of 6.3% over all the considered cancers, ranging from 0.4% (Naples, Italy) to 12.9% (Iceland). The proportion of multiple tumours varied greatly by type of tumour, being higher for those with high incidence and long survival (breast, prostate and colon-rectum). Five-year relative survival was lower when including patients with multiple cancers. For all cancers combined the average difference was -0.4 percentage points in women and -0.7 percentage points in men, and was greater for older registries. Inclusion of multiple tumours led to lower survival in 44 out of 45 cancer sites analysed, with the greatest differences found for larynx (-1.9%), oropharynx (-1.5%), and penis (-1.3%). Including multiple primary tumours in survival estimates for international comparison is advisable because it reduces the bias due to different observation periods, age, registration quality and completeness of registration. The general effect of inclusion is to reduce survival estimates by a variable amount depending on the proportion of multiple primaries and cancer site. (C) 2008 Elsevier Ltd. All rights reserved.

International audience; OBJECTIVE: To examine whether childhood brain tumors (CBTs) are associated with a family history of brain tumors or other cancers in an international case-control study. METHODS: Cancers in children's first- and second-degree relatives were ascertained by interview with parents of 620 children with astroglial tumors, 255 with primitive neuroectodermal tumors, 324 with other CBTs, and 2,218 controls from Australia, Canada, France, Israel, Italy, Spain, and the US. These were used with histories of neurofibromatosis or tuberous sclerosis to exclude in subanalyses children with Li-Fraumeni or other hereditary syndromes predisposing to brain tumors. RESULTS: A first- or second-degree relative of 4% of children with astroglial tumors, 6% with PNET, 5% with other CBTs, and 5% of controls had had a brain tumor. Any potential differences were statistically non-significant, including when focusing on relatives diagnosed in childhood. In the US, where anatomical sites of relatives' other cancers were known, CBT occurrence was not associated with any other specific site. Results were not markedly altered by exclusion of children with hereditary syndromes. CONCLUSION: Consistent with most prior studies using these methods, we observed no strong relationship between CBT occurrence and cancers in family members.

This paper describes the incidence and survival of childhood central nervous system (CNS) tumours in Europe for the period 1978–1997. A total of 19,531 cases, aged 0–14 years, from the ACCIS database were analysed by five regions: the British Isles, East, North, South, and West. Overall age-standardised incidence rate (ASR) of CNS tumours in Europe (1988–1997) was 29.9 per million, with the highest rates in the North. Astrocytoma (ASR = 11.8), primitive neuroectodermal tumours (PNET) (ASR = 6.5) and ependymoma (ASR = 3.4) were the most frequent types. Incidence increased significantly during 1978–1997, on average by 1.7% per year. Diagnostic methods may partially explain incidence rates and trends, although a role of variations in risk factors cannot be excluded. Overall 5-year survival was 64% and varied between 72% in the North and 53% in the East. PNET had the poorest prognosis (49%) and astrocytoma the best (75%). Survival has improved by 29% since late 1970s. The positive trends were seen in all regions, although the interregional differences persisted, as a reflection of the different healthcare systems.

To access publisher's full text version of this article. Please click on the hyperlink in Additional Links field. Survival and cure rates for childhood cancers in Europe have greatly improved over the past 40 years and are mostly good, although not in all European countries. The EUROCARE-5 survival study estimates survival of children diagnosed with cancer between 2000 and 2007, assesses whether survival differences among European countries have changed, and investigates changes from 1999 to 2007. We analysed survival data for 157,499 children (age 0-14 years) diagnosed between Jan 1, 1978 and Dec 31, 2007. They came from 74 population-based cancer registries in 29 countries. We calculated observed, country-weighted 1-year, 3-year, and 5-year survival for major cancers and all cancers combined. For comparison between countries, we used the corrected group prognosis method to provide survival probabilities adjusted for multiple confounders (sex, age, period of diagnosis, and, for all cancers combined without CNS cancers, casemix). Age-adjusted survival differences by area and calendar period were calculated with period analysis and were given for all cancers combined and the major cancers. We analysed 59,579 cases. For all cancers combined for children diagnosed in 2000-07, 1-year survival was 90.6% (95% CI 90.2-90.9), 3-year survival was 81.0 % (95% CI 80.5-81.4), and 5-year survival was 77.9% (95% CI 77.4-78.3). For all cancers combined, 5-year survival rose from 76.1% (74.4-77.7) for 1999-2001, to 79.1% (77.3-80.7) for 2005-07 (hazard ratio 0.973, 95% CI 0.965-0.982, p

Objective: To evaluate the role of parental occupations in the etiology of childhood brain tumors (CBT).

An international case-control study of primary pediatric brain tumors included interviews with mothers of cases diagnosed from 1976-1994 and mothers of population controls. Data are available on maternal vitamin use during pregnancy for 1051 cases and for 1919 controls in eight geographic areas of North America, Europe and Israel. While risk estimates varied by study center, combined results suggest that maternal supplementation for two trimesters may decrease risk of brain tumor [odds ratio (OR)=0.7; 95% confidence interval (CI)=0.5-0.9], with a trend toward less risk with longer duration of use (P trend= 0.0007). The greatest risk reduction was among children diagnosed under 5 years of age whose mothers used supplements during all three trimesters (OR=0.5; CI=0.3- 0.8). This effect did not vary by histology and was seen for supplementation during pregnancy rather than during the month before pregnancy or while breast feeding. These findings are largely driven by data from the US, where most mothers took vitamins. The proportion of control mothers who took vitamins during pregnancy varied tremendously, from 3% in Israel and in France through 21% in Italy, 33% in Canada, 52% in Spain to 86-92% at the three US centers. The composition of the various multivitamin compounds taken also varied: daily dose of vitamin C ranged from 0 up to 600 mg; vitamin E from 0 to 70 mg; vitamin A from 0 to 30,000 IU and folate from 0 to 2000 mg. Mothers also took individual micronutrient supplements (e.g., vitamin C tablets), but most mothers who took these also took multivitamins, making it impossible to determine potential independent effects of these micronutrients.

The role of parental occupational exposure in childhood brain tumorswas investigated in a population-based case-control study grouping 251 casesand 601 controls from three European centers: Milan (Italy), Paris (France),and Valencia (Spain). Parental occupational exposure to solvents andpolycyclic aromatic hydrocarbons (PAH) during the five-year period beforebirth was estimated using a job-exposure matrix developed earlier in the samecountries. Odds ratios (OR) of brain tumors for each occupation andoccupational exposure were estimated by logistic regression, adjusting forchild’s age, gender, exposure to tobacco smoke and ionizing radiation,mother’s age and years of schooling, and center. The risk of childhood braintumors rose when fathers worked in agriculture (OR = 2.2, 95 percentconfidence interval [CI] = 1.0-4.7) and motor-vehicle-related occupations. Inthe latter group, the risk increased for primitive neuroectodermal tumors inparticular (OR = 2.7, CI = 1.1-6. 6). Astroglial tumors were more frequentamong children of mothers in health services (OR = 2.2, CI = 1.0-4.9).Paternal exposure to PAHs was associated with an increased, but notdose-related, risk of primitive neuroectodermal tumors (OR = 2.0, CI =1.0-4.0), and maternal exposure to solvents at a high level was associatedwith an increased risk of both astroglial (OR = 2.3, CI = 0.9-5.8) andprimitive neuroectodermal tumors (OR = 3.2, CI = 1.0-10.3).

Persons with Down syndrome (DS) uniquely have an increased frequency of leukemias but a decreased total frequency of solid tumors. The distribution and frequency of specific types of brain tumors have never been studied in DS. We evaluated the frequency of primary neural cell embryonal tumors and gliomas in a large international data set. The observed number of children with DS having a medulloblastoma, central nervous system primitive neuroectodermal tumor (CNS-PNET) or glial tumor was compared to the expected number. Data were collected from cancer registries or brain tumor registries in 13 countries of Europe, America, Asia and Oceania. The number of DS children with each category of tumor was treated as a Poisson variable with mean equal to 0.000884 times the total number of registrations in that category. Among 8,043 neural cell embryonal tumors (6,882 medulloblastomas and 1,161 CNS-PNETs), only one patient with medulloblastoma had DS, while 7.11 children in total and 6.08 with medulloblastoma were expected to have DS. (p 0.016 and 0.0066 respectively). Among 13,797 children with glioma, 10 had DS, whereas 12.2 were expected. Children with DS appear to be specifically protected against primary neural cell embryonal tumors of the CNS, whereas gliomas occur at the same frequency as in the general population. A similar protection against neuroblastoma, the principal extracranial neural cell embryonal tumor, has been observed in children with DS. Additional genetic material on the supernumerary chromosome 21 may protect against embryonal neural cell tumor development.

The aim of this study was to estimate the incidence of leukaemias and lymphomas in children according to the International Classification of Childhood Cancer third edition (ICCC-3) in the population covered by the Girona, Valencia, and Zaragoza population-based cancer registries and compare it with the incidence rates in other European countries. All haematological malignancies (HMs) registered between 1993 and 2002 in children below 15 years of age were included in the study. Pathological and haematological diagnoses were reviewed, recoded according to International Classification of Diseases for Oncology-3 and reclassified on the basis of ICCC-3. Sex and age-adjusted incidence rates were calculated, using the world population as standard. Five hundred and seventy-one HMs were registered in the Girona, Valencia and Zaragoza Cancer Registries during the study period. According to ICCC-3, precursor cell leukaemias were the most frequent HMs in children and constituted 60% of all HMs (an age-adjusted incidence rate of 42.7 per million children-years). The second most frequent childhood HM was Hodgkin lymphoma (11.2% of all HMs), yielding an age-adjusted standardized incidence rate of 6.3 per million children-years. With regard to myeloid lineage, acute myeloid leukaemias were the most frequent with a rate of 7.9 per million children-years. The standardized incidence rates for lymphoid leukaemia (1.19) and Burkitt lymphoma (3.94) were statistically higher than the rates observed in Europe. Compared with European data, Spain has a high incidence of lymphoid leukaemias and lymphomas. In particular, a high incidence of Burkitt lymphoma was observed. The causes of this geographical variation are still unknown.

International audience; Adenoid cystic carcinoma (ACC) of salivary gland origin is rare. The EUROCARE data provide a good opportunity to study the survival of this uncommon cancer in a large population. A total of 2611 cases, aged 15 to 99 years, diagnosed between 1983 and 1994 with primary salivary gland ACC were analyzed. Thirty-two population based cancer registries from seventeen countries participating in EUROCARE contributed the data. Relative survival by sex, age, period of diagnosis, region, site and stage, and the adjusted relative excess risk (RER) of death were estimated. Survival since diagnosis was 94%, 78% and 65% at 1, 5 and ten years, respectively. Ten-year survival was best (69%) in patients of the youngest age group (15-54 years) and from Northern Europe (69%). In the UK was higher (65%) than in Western (62%) and Eastern (56%) Europe. ACCs in nasal cavity (RER 2.6), pharynx (RER 3.5) and larynx and bronchus (RER 3.9) had a worse prognosis compared to those of oral cavity. A strong effect of stage at diagnosis on RERs and some worsening of survival at five years over time (80% in 1983-1985, 76% in 1992-1994) were also evident. The findings of the present study, as those from clinical studies, confirm the important impact of primary site and stage at diagnosis on survival. Furthermore, we could demonstrate that survival for ACC did not improve over time and that cases from Eastern countries had a significant worse prognosis. Improvements in the disease detection in its early stage and international collaborative research should be encouraged.

Background 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. Methods 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. Results 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. Conclusion 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.

International audience; BACKGROUND: The survival of patients with Hodgkin disease (HD) varies markedly across Europe and generally is shorter than the survival of patients in the U.S. To investigate these differences, the authors compared population-based HD survival in relation to morphologic type among populations in Europe and the U.S. METHODS: The authors analyzed 6726 patients from 37 cancer registries that participated in EUROCARE-3 and 3442 patients from 9 U.S. Surveillance, Epidemiology, and End Results (SEER) registries. Patients were diagnosed during 1990 to 1994 and were followed for at least 5 years. The European registries were grouped into EUROCARE West, EUROCARE UK, and EUROCARE East. Morphologic groups were nodular sclerosis, mixed cellularity, lymphocyte depletion, lymphocyte predominance, and not otherwise specified (NOS). The influence of morphology on geographic differences in 5-year relative survival was explored by using multiple regression analysis. RESULTS: In the model that was adjusted by age, gender, and years since diagnosis, the relative excess risk (RER) of death was 0.93 (95% confidence interval [95% CI], 0.81-1.05) in EUROCARE West, 1.15 (95% CI, 1.04-1.28) in EUROCARE UK, and 1.39 (95% CI, 1.21-1.60) in EUROCARE East (compared with the SEER data). When morphology was included, EUROCARE UK and SEER no longer differed (RER, 1.06; 95% CI, 0.95-1.18). Morphology distribution varied markedly across Europe and much less in the U.S., with nodular sclerosis less common in Europe (45.9%) than the U.S. (61.7%). The RER data showed that patients who had lymphocyte depletion, NOS, and mixed cellularity had a significantly worse prognoses compared with patients who had nodular sclerosis, whereas patients who had lymphocyte predominance had the best prognosis. CONCLUSIONS: The current results provide population-based evidence that morphology strongly influences the prognosis of patients with HD. However differences in the morphologic case mix explains only some of the geographic variations observed in survival.

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.

A total of 1218 cases of childhood brain tumours (CBT) and 2223 control subjects from the general population were included in a population-based case-control study conducted in nine centres in seven countries. Mothers were asked about farm- or agriculture-related exposures. Significantly elevated odds ratios (OR) for CBT were associated with children's personal and maternal prenatal exposure while living on a farm with pigs (child OR = 1.7, mother OR = 2.3), horses (child OR = 1.6, mother OR = 1.8), dogs (child OR = 1.5, mother OR = 1.5) and cats (child OR = 1.5, mother OR = 1.7). Children who were exposed to pigs, horses and cats combined, while living on a farm, had a threefold elevated OR for CBT. Increased ORs for primitive neuroectodermal tumours (PNET) were associated with children's farm exposure to dogs (OR = 1.9) and cats (OR = 2.2), and maternal farm exposure to pigs (OR = 4.2). The OR for CBT was elevated (OR = 2.3) for children of mothers who had preconception/prenatal farm- or agriculture-related employment involving potential contact with animals, relative to no farm- or agriculture-related employment. In particular, increased ORs for CBT were observed for children of mothers who were employed as general farmers (OR = 4.1) or general farm workers (OR = 3.8). During the 5 years preceding the index child's birth, maternal exposures were related to CBT, relative to no maternal exposure to agricultural chemicals or animal products: fertilisers (OR = 1.8), pesticides (OR = 2.0), animal manure (OR = 2.0) and unprocessed wool (OR = 3.0). Our findings suggest that various farm-related exposures are positively associated with CBT and warrant further investigation into the public health importance of these associations.

The EUROCARE-3 CD-ROM has been developed to provide more detailed data with respect to those published in the monograph. The CD-ROM provides estimates of age-specific and age-standardised survival figures, cumulative and interval-specific survival, observed and relative survival for 47 cancer sites or combinations of sites, based on >4 million adult cancer patients diagnosed from 1983 to 1994 and reported from 56 European cancer registries. In addition, the CD-ROM provides observed survival proportions for 25 childhood cancer entities based on 23,000 young patients diagnosed from 1990 to 1994. Survival indicators, corresponding standard errors and confidence intervals can be selected according to cancer site, registry or country, sex, age class and disease duration. Basic graphical display and export facilities have also been provided. As an example of how to use this CD-ROM, this paper will report a descriptive analysis of relative survival patterns for all cancers combined, by age, sex and country. The EUROCARE-3 CD-ROM can be ordered free of charge or directly downloaded at http://www.eurocare.it.

The EUROCLUS study assembled incidence data for 13,551 cases of childhood leukaemia (CL) diagnosed between 1980 and 1989 in 17 countries (or regions of countries). These were referenced by location at diagnosis to small census areas of which there were 25,723 in the study area. Population counts, surface area and, hence, population density were available for all these small areas. Previous analyses have shown limited extra-Poisson variation (EPV) of case counts within small areas; this is most pronounced in areas of intermediate population density (150-499 persons/km2). In this study, the data set was examined in more detail for evidence that variations in incidence and EPV of CL are associated with population density. Incidence showed a curvilinear association with population density and was highest in areas which were somewhat more densely populated (500-750 persons/km2), where the incidence rate ratio relative to areas having > or = 1000 persons/km2 was 1.16 (95% confidence interval 1.07-1.26) and the P value for quadratic trend across eight strata of population density was 0.02. Incidence in these areas is uniformly elevated and showed no evidence of heterogeneity (i.e. EPV). Statistically significant evidence of EPV was evident amongst some of the areas previously classified as intermediate density areas (specifically, those with a density of 250-499 persons/km2, P < 0.001 for CL). These results were interpreted in terms of the current aetiological hypotheses for CL which propose that exposure to localised epidemics of one or more common infectious agent may contribute to the development of leukaemia. They suggest that such epidemics arise regularly in moderately densely populated areas and also sporadically in areas which are somewhat less densely populated. Although other interpretations are possible, these results may assist in the identification of characteristics which infectious agents must possess if direct or indirect causes of CL.

ResumenObjetivosDescribir un procedimiento sistemático para la recuperación exhaustiva de trabajos publicados en revistas españolas sobre evaluación de los sistemas de información clínicos y sanitarios (1983–1992) y analizar los términos útiles, el aporte y los fallos de las fuentes.MétodosComo fuentes para la identificación de trabajos se ha utilizado la base de datos Índice Médico Español (IME), los sumarios de revistas y la bibliografía de los trabajos recuperados. Como variables de análisis se han utilizado los términos coincidentes entre perfil de búsqueda, títulos y palabras clave; Índices de aporte, aporte específico y solapamiento; y fallos del usuario, actualización y cobertura.ResultadosSe han recuperado 94 trabajos, de los que 83 tienen al menos un término sobre el tema de interés. En cuanto al aporte, IME proporcionó 74 trabajos, los sumarios 75 y la bibliografía 33. El aporte específico del IME fue de 16 trabajos, el de los sumarios de 12, y el de la bibliografía de 2. El solapamiento entre las fuentes fue 25,5%.ConclusiónLas tres fuentes utilizadas son complementarias. La recuperación exhaustiva de la literatura requiere usar sistemáticamente fuentes como los sumarios de las revistas o la bibliografía de los trabajos, donde la percepción de los contenidos de un trabajo es menos dependiente de los términos presentes en sus títulos.SummaryObjectivesTo describe a systematic procedure for exhaustive retrieval of Spanish articles about evaluation or health information systems (1983–1992); to analize the useful terms, the item contribution and the retrieval failures of the sources.MethodsAs sources for identification of articles, data base Índice Médico Español (IME), contents of journals and references of the retrieved items have been used. As analysis variables, coincidence of terms in search profile, item's titles and key words; item contribution, specific contribution and overlap indexes; hindsight analysis of failures.Results94 items have been retrieved, 83 of them have, at least one term related with the subject. Referred to sources contribution, IME gives 74 items; journal contents 75 and references 33. Specific contribution of IME was 16 items, contents 12 and references 2. Overlap between sources was 25.5%.ConclusionThe three sources are complementary. Complete retrieval of the literature requires systematic use of the abstracts and references of the papers, where the perception of its contents are less depedent of the terms used in the titles.

The EUROCLUS project included information on residence at diagnosis for 13351 cases of childhood leukaemia diagnosed in the period 1980-89 in defined geographical regions in 17 countries. A formal algorithm permits identification of small census areas as containing case excesses. The present analysis examines spatial-temporal patterns of the cases (n = 970) within these clustered areas. The objectives were, first, to compare these results with those from an analysis conducted for UK data for the period 1966-83, and, second, to extend them to consider infant leukaemias. A modification of the Knox test investigates, within the small areas, temporal overlap between cases in a subgroup of interest at a putative critical time and all other cases at any time between birth and diagnosis. Critical times were specified in advance as follows: for cases of acute lymphoblastic leukaemia aged 2-4 years, the 18-month period preceding diagnosis; for cases of total leukaemia aged 5-14 years, 1 year before to 1 year after birth; and for infant cases (diagnosed < 1 year), 1 year before to 6 months after birth. Each of the analyses found evidence of excess space-time overlap compared with that expected; these were 10% (P = 0.005), 15% (P= 0.0002) and 26% (P= 0.03) respectively. The results are interpreted in terms of an infectious origin of childhood leukaemia.

The interpretation of reports of clusters of childhood leukaemia is difficult, first because little is known about the causes of the disease, and second because there is insufficient information on whether cases show a generalized tendency to cluster geographically. The EUROCLUS project is a European collaborative study whose primary objective is to determine whether the residence locations of cases at diagnosis show a general tendency towards spatial clustering. The second objective is to interpret any patterns observed and, in particular, to see if clustering can be explained in terms of either infectious agents or environmental hazards as aetiological agents. The spatial distribution of 13351 cases of childhood leukaemia diagnosed in 17 countries between 1980 and 1989 has been analysed using the Potthoff-Whittinghill method. The overall results show statistically significant evidence of clustering of total childhood leukaemia within small census areas (P=0.03) but the magnitude of the clustering is small (extra-Poisson component of variance (%) = 1.7 with 90% confidence interval 0.2-3.1). The clustering is most marked in areas that have intermediate population density (150-499 persons km[-2]). It cannot be attributed to any specific age group at diagnosis or cell type and involves spatial aggregation of cases of different ages and cell types. The results indicate that intense clusters are a rare phenomenon that merit careful investigation, although aetiological insights are more likely to come from investigation of large numbers of cases. We present a method for detecting clustering that is simple and readily available to cancer registries and similar groups.