Incidence of childhood cancer in Latin America and the Caribbean: coverage, patterns, and time trends.

Objective: To provide a comprehensive overview of geographical patterns (2001-2010) and time trends (1993-2012) of cancer incidence in children aged 0-19 years in Latin America and the Caribbean (LAC) and interpret the findings in the context of global patterns. Methods: Geographical variations in 2001-2010 and incidence trends over 1993-2012 in the population of LAC younger than 20 years were described using the database of the third volume of the International Incidence of Childhood Cancer study containing comparable data. Age-specific incidence per million person-years (ASR) was calculated for population subgroups and age-standardized (WSR) using the world standard population. Results: Overall, 36 744 unique cases were included in this study. In 2001-2010 the overall WSR in age 0-14 years was 132.6. The most frequent were leukemia (WSR 48.7), central nervous system neoplasms (WSR 23.0), and lymphoma (WSR 16.6). The overall ASR in age group 15-19 years was 152.3 with lymphoma ranking first (ASR 30.2). Incidence was higher in males than in females, and higher in South America than in Central America and the Caribbean. Compared with global data LAC incidence was lower overall, except for leukemia and lymphoma at age 0-14 years and the other and unspecified tumors at any age. Overall incidence at age 0-19 years increased by 1.0% per year (95% CI [0.6, 1.3]) over 1993-2012. The included registries covered 16% of population aged 0-14 years and 10% of population aged 15-19 years. Conclusions: The observed patterns provide a baseline to assess the status and evolution of childhood cancer occurrence in the region. Extended and sustained support of cancer registration is required to improve representativeness and timeliness of data for childhood cancer control in LAC.

Incidence of childhood cancer in Latin America and the Caribbean: coverage, patterns, and time trends / Incidencia del cáncer infantil en América Latina y el Caribe: cobertura de los registros, patrones y tendencias a lo largo del tiempo / Incidência de câncer infantil na América Latina e no Caribe: cobertura, padrões e tendências temporais

ABSTRACT Objective. To provide a comprehensive overview of geographical patterns (2001-2010) and time trends (1993-2012) of cancer incidence in children aged 0-19 years in Latin America and the Caribbean (LAC) and interpret the findings in the context of global patterns. Methods. Geographical variations in 2001-2010 and incidence trends over 1993-2012 in the population of LAC younger than 20 years were described using the database of the third volume of the International Incidence of Childhood Cancer study containing comparable data. Age-specific incidence per million person-years (ASR) was calculated for population subgroups and age-standardized (WSR) using the world standard population. Results. Overall, 36 744 unique cases were included in this study. In 2001-2010 the overall WSR in age 0-14 years was 132.6. The most frequent were leukemia (WSR 48.7), central nervous system neoplasms (WSR 23.0), and lymphoma (WSR 16.6). The overall ASR in age group 15-19 years was 152.3 with lymphoma ranking first (ASR 30.2). Incidence was higher in males than in females, and higher in South America than in Central America and the Caribbean. Compared with global data LAC incidence was lower overall, except for leukemia and lymphoma at age 0-14 years and the other and unspecified tumors at any age. Overall incidence at age 0-19 years increased by 1.0% per year (95% CI [0.6, 1.3]) over 1993-2012. The included registries covered 16% of population aged 0-14 years and 10% of population aged 15-19 years. Conclusions. The observed patterns provide a baseline to assess the status and evolution of childhood cancer occurrence in the region. Extended and sustained support of cancer registration is required to improve representativeness and timeliness of data for childhood cancer control in LAC.

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RESUMO Objetivo. Apresentar uma visão abrangente dos padrões geográficos (2001 a 2010) e das tendências temporais (1993 a 2012) da incidência de câncer em crianças e jovens de 0 a 19 anos na América Latina e no Caribe (ALC) e interpretar os resultados no contexto de padrões mundiais. Métodos. Foram descritas variações geográficas de 2001 a 2010 e tendências de incidência de 1993 a 2012 na população com menos de 20 anos da ALC usando informações comparáveis da base de dados do terceiro volume do estudo International Incidence of Childhood Cancer. Foram calculadas taxas de incidência específica por idade por milhão de pessoas-ano (ASR, na sigla em inglês) para subgrupos populacionais e taxas padronizadas por idade usando a população padrão mundial (WSR, na sigla em inglês). Resultados. No total, foram incluídos 36 744 casos únicos. No período de 2001 a 2010, a WSR para todos os tumores combinados na faixa etária de 0 a 14 anos foi de 132,6. Os diagnósticos mais frequentes foram leucemia (WSR de 48,7), neoplasias do sistema nervoso central (WSR de 23,0) e linfoma (WSR de 16,6). A ASR para todos os tumores combinados na faixa etária de 15 a 19 anos foi de 152,3, e a maior taxa foi a de linfoma (ASR de 30,2). A incidência foi maior no sexo masculino do que no sexo feminino e maior na América do Sul do que na América Central e no Caribe. De modo geral, em comparação com as estimativas mundiais, a incidência na ALC foi menor, exceto para leucemia e linfoma entre 0 e 14 anos e para outros tumores e tumores não especificados em qualquer idade. A taxa de incidência na faixa etária de 0 a 19 anos aumentou em 1,0% ao ano (IC de 95% [0,6, 1,3]) entre 1993 e 2012. Os registros incluídos cobriam 16% da população de 0 a 14 anos e 10% da população de 15 a 19 anos. Conclusões. Os padrões observados servem de referência para avaliar o status e a evolução da ocorrência de câncer infantil na região. É necessário garantir um apoio ampliado e consistente aos registros de câncer para aprimorar a representatividade e a disponibilidade das informações em tempo adequado para o controle do câncer infantil na ALC.

A new method of estimating prevalence of childhood cancer survivors (POCCS): example of the 20-year prevalence in The Netherlands.

BACKGROUND: Estimating the number of childhood cancer survivors is crucial for cancer control, including clinical guidelines. To compare estimates across countries despite data sharing restrictions, we propose a new method of computing limited-duration prevalence of childhood cancer survivors (POCCS) using aggregated data. METHODS: We developed a Markov model that simulates, for each calendar year and birth cohort in a population, the proportion of individuals in the following health states: healthy, newly diagnosed with cancer, surviving with cancer, and deceased. Transitions between health states were informed using annual sex- and age-specific incidence rates, conditional 1-year net survival probabilities from the Netherlands Cancer Registry (1989-2011), and annual mortality probability by sex and age group for The Netherlands from the Human Mortality Database. Applying a Markov model, we computed 20-year prevalence of childhood cancer survivors. The resulting POCCS estimates, stratified by sex, were compared with SEER*Stat estimates derived from individual cancer records from the same registry. RESULTS: In 2011, POCCS predicted 654 males [95% confidence interval (95% CI): 637-672] and 539 females (95% CI: 523-555) per million persons living in The Netherlands after childhood cancer diagnosed within the previous 20 years. Using SEER*Stat, the 20-year prevalence was 665 males (95% CI: 647-683) and 544 females (95% CI: 529-560) per million persons on 1 July 2011. CONCLUSIONS: Using the POCCS model and aggregated cancer data, our estimates of childhood cancer survivors limited-duration prevalence were consistent with those computed by a standard method requiring individual cancer records. The POCCS method provides relevant information for planning follow-up and care for childhood cancer survivors.

Subsequent cancers within 5 years from initial diagnosis of childhood cancer. Patterns and risks in the population of Great Britain.

BACKGROUND: Patterns and risks of subsequent primary tumours (SPTs) among long-term survivors of childhood cancer have been extensively described, but much less is known about early SPTs (ESPTs) occurring within 5 years after initial diagnosis. PROCEDURE: We carried out a population-based study of ESPTs following childhood cancer throughout Britain, using the National Registry of Childhood Tumours. The full study series comprised all ESPTs occurring among 56,620 children whose initial cancer diagnosis was in the period 1971-2010. Frequencies of ESPT were calculated for the entire cohort. For analyses of risk, follow-up began 92 days after initial diagnosis. RESULTS: ESPT developed in 0.4% of children overall, 0.52% of those initially diagnosed at age less than 1 year and 0.38% of those diagnosed at age 1-14 years. Standardised incidence ratio (SIR) was 7.7 (95% confidence interval [CI]: 6.7-8.9), overall 9.5 (95% CI: 7.1-12.5) for children initially diagnosed in 1981-1990 and 6.5-7.5 for those from earlier and later decades. SIR by type of first cancer ranged from 4.4 (95% CI: 1.8-9.1) for Wilms tumour to 13.1 (95% CI: 7.7-21.0) for non-Hodgkin lymphoma. SIR by type of ESPT ranged from 2.0 (95% CI: 1.0-3.4) for acute lymphoblastic leukaemia to 66.6 (95% CI: 52.3-83.6) for acute myeloid leukaemia. Predisposition syndromes were known to be implicated in 21% of children with ESPT and suspected in another 5%. CONCLUSIONS: This study provides an overview of the patterns and risks of ESPTs in a large population where many children received therapy that is still in widespread use. Further research will be needed to monitor and understand changes in risk as childhood cancer treatment continues to evolve.

Incidence, prevalence and survival in patients with Langerhans cell histiocytosis: A national registry study from England, 2013-2019.

This analysis is the largest population-based study to date to provide contemporary and comprehensive epidemiological estimates of all third edition of the International Classification of Diseases for Oncology (ICD-O-3) coded Langerhans cell histiocytosis (LCH) from England. People of all ages were identified from the National Cancer Registration Dataset using ICD-O-3 morphologies 9751-9754 for neoplasms diagnosed in 2013-2019. A total of 658 patients were identified, of whom 324 (49%) were children aged <15 years. The age-standardised incidence rate was 4.46 (95% confidence interval [CI] 3.99-4.98) per million children and 1.06 (95% CI 0.94-1.18) per million adults aged ≥15 years. Prevalence of LCH was 9.95 (95% CI 9.14-10.81) per million persons at the end of 2019. The 1-year overall survival (OS) was 99% (95% CI 97%-100%) for children and 90% (95% CI 87%-93%) for adults. Those aged ≥60 years had poorer OS than those aged <15 years (hazard ratio [HR] 22.12, 95% CI 7.10-68.94; p < 0.001). People in deprived areas had lower OS than those in the least deprived areas (HR 5.36, 95% CI 1.16-24.87; p = 0.03). There will inevitably be other environmental factors and associations yet to be identified, and the continued standardised data collection will allow further evaluation of data over time. This will be increasingly important with developments in LCH management following the large collaborative international trials such as LCH IV.

Does the morphology of cutaneous melanoma help to explain the international differences in survival? Results from 1 578 482 adults diagnosed during 2000-2014 in 59 countries (CONCORD-3).

BACKGROUND: CONCORD-3 highlighted wide disparities in population-based 5-year net survival for cutaneous melanoma during 2000-2014. Clinical evidence suggests marked international differences in the proportion of lethal acral and nodular subtypes of cutaneous melanoma. OBJECTIVES: We aimed to assess whether the differences in morphology may explain global variation in survival. METHODS: Patients with melanoma were grouped into the following seven morphological categories: malignant melanoma, not otherwise specified (International Classification of Diseases for Oncology, third revision morphology code 8720), superficial spreading melanoma (8743), lentigo maligna melanoma (8742), nodular melanoma (8721), acral lentiginous melanoma (8744), desmoplastic melanoma (8745) and other morphologies (8722-8723, 8726-8727, 8730, 8740-8741, 8746, 8761, 8770-8774, 8780). We estimated net survival using the nonparametric Pohar Perme estimator, correcting for background mortality by single year of age, sex and calendar year in each country or region. All-ages survival estimates were standardized using the International Cancer Survival Standard weights. We fitted a flexible parametric model to estimate the effect of morphology on the hazard of death. RESULTS: Worldwide, the proportion of nodular melanoma ranged between 7% and 13%. Acral lentiginous melanoma accounted for less than 2% of all registrations but was more common in Asia (6%) and Central and South America (7%). Overall, 36% of tumours were classified as superficial spreading melanoma. During 2010-2014, age-standardized 5-year net survival for superficial spreading melanoma was 95% or higher in Oceania, North America and most European countries, but was only 71% in Taiwan. Survival for acral lentiginous melanoma ranged between 66% and 95%. Nodular melanoma had the poorest prognosis in all countries. The multivariable analysis of data from registries with complete information on stage and morphology found that sex, age and stage at diagnosis only partially explain the higher risk of death for nodular and acral lentiginous subtypes. CONCLUSIONS: This study provides the broadest picture of distribution and population-based survival trends for the main morphological subtypes of cutaneous melanoma in 59 countries. The poorer prognosis for nodular and acral lentiginous melanomas, more frequent in Asia and Latin America, suggests the need for health policies aimed at specific populations to improve awareness, early diagnosis and access to treatment. What is already known about this topic? The histopathological features of cutaneous melanoma vary markedly worldwide. The proportion of melanomas with the more aggressive acral lentiginous or nodular histological subtypes is higher in populations with predominantly dark skin than in populations with predominantly fair skin. What does this study add? We aimed to assess the extent to which these differences in morphology may explain international variation in survival when all histological subtypes are combined. This study provides, for the first time, international comparisons of population-based survival at 5 years for the main histological subtypes of melanoma for over 1.5 million adults diagnosed during 2000-2014. This study highlights the less favourable distribution of histological subtypes in Asia and Central and South America, and the poorer prognosis for nodular and acral lentiginous melanomas. We found that later stage at diagnosis does not fully explain the higher excess risk of death for nodular and acral lentiginous melanoma compared with superficial spreading melanoma.

International variation in childhood cancer mortality rates from 2001 to 2015: Comparison of trends in the International Cancer Benchmarking Partnership countries.

Despite improved survival rates, cancer remains one of the most common causes of childhood death. The International Cancer Benchmarking Partnership (ICBP) showed variation in cancer survival for adults. We aimed to assess and compare trends over time in cancer mortality between children, adolescents and young adults (AYAs) and adults in the six countries involved in the ICBP: United Kingdom, Denmark, Australia, Canada, Norway and Sweden. Trends in mortality between 2001 and 2015 in the six original ICBP countries were examined. Age standardised mortality rates (ASR per million) were calculated for all cancers, leukaemia, malignant and benign central nervous system (CNS) tumours, and non-CNS solid tumours. ASRs were reported for children (age 0-14 years), AYAs aged 15 to 39 years and adults aged 40 years and above. Average annual percentage change (AAPC) in mortality rates per country were estimated using Joinpoint regression. For all cancers combined, significant temporal reductions were observed in all countries and all age groups. However, the overall AAPC was greater for children (-2.9; 95% confidence interval = -4.0 to -1.7) compared to AYAs (-1.8; -2.1 to -1.5) and adults aged >40 years (-1.5; -1.6 to -1.4). This pattern was mirrored for leukaemia, CNS tumours and non-CNS solid tumours, with the difference being most pronounced for leukaemia: AAPC for children -4.6 (-6.1 to -3.1) vs AYAs -3.2 (-4.2 to -2.1) and over 40s -1.1 (-1.3 to -0.8). AAPCs varied between countries in children for all cancers except leukaemia, and in adults over 40 for all cancers combined, but not in subgroups. Improvements in cancer mortality rates in ICBP countries have been most marked among children aged 0 to 14 in comparison to 15 to 39 and over 40 year olds. This may reflect better care, including centralised service provision, treatment protocols and higher trial recruitment rates in children compared to older patients.

COVID-19 in children with haematological malignancies.

BACKGROUND: Children with cancer are not at increased risk of severe SARS-CoV-2 infection; however, adults with haematological malignancies have increased risk of severe infections compared with non-haematological malignancies. METHODS: We compared patients with haematological and non-haematological malignancies enrolled in the UK Paediatric Coronavirus Cancer Monitoring Project between 12 March 2020 and 16 February 2021. Children who received stem cell transplantation were excluded. RESULTS: Only 2/62 patients with haematological malignancy had severe/critical infections, with an OR of 0.5 for patients with haematological compared with non-haematological malignancies. INTERPRETATION: Children with haematological malignancies are at no greater risk of severe SARS-CoV-2 infection than those with non-haematological malignancies.

The histology of brain tumors for 67 331 children and 671 085 adults diagnosed in 60 countries during 2000-2014: a global, population-based study (CONCORD-3).

BACKGROUND: Global variations in survival for brain tumors are very wide when all histological types are considered together. Appraisal of international differences should be informed by the distribution of histology, but little is known beyond Europe and North America. METHODS: The source for the analysis was the CONCORD database, a program of global surveillance of cancer survival trends, which includes the tumor records of individual patients from more than 300 population-based cancer registries. We considered all patients aged 0-99 years who were diagnosed with a primary brain tumor during 2000-2014, whether malignant or nonmalignant. We presented the histology distribution of these tumors, for patients diagnosed during 2000-2004, 2005-2009, and 2010-2014. RESULTS: Records were submitted from 60 countries on 5 continents, 67 331 for children and 671 085 for adults. After exclusion of irrelevant morphology codes, the final study population comprised 60 783 children and 602 112 adults. Only 59 of 60 countries covered in CONCORD-3 were included because none of the Mexican records were eligible. We defined 12 histology groups for children, and 11 for adults. In children (0-14 years), the proportion of low-grade astrocytomas ranged between 6% and 50%. Medulloblastoma was the most common subtype in countries where low-grade astrocytoma was less commonly reported. In adults (15-99 years), the proportion of glioblastomas varied between 9% and 69%. International comparisons were made difficult by wide differences in the proportion of tumors with unspecified histology, which accounted for up to 52% of diagnoses in children and up to 65% in adults. CONCLUSIONS: To our knowledge, this is the first account of the global histology distribution of brain tumors, in children and adults. Our findings provide insights into the practices and the quality of cancer registration worldwide.

Global patterns and trends in incidence and mortality of thyroid cancer in children and adolescents: a population-based study.

BACKGROUND: There has been a considerable increase in thyroid cancer incidence among adults in several countries in the past three decades, attributed primarily to overdiagnosis. We aimed to assess global patterns and trends in incidence and mortality of thyroid cancer in children and adolescents, in view of the increased incidence among adults. METHODS: We did a population-based study of the observed incidence (in 49 countries and territories) and mortality (in 27 countries) of thyroid cancer in children and adolescents aged 0-19 years using data from the International Incidence of Childhood Cancer Volume 3 study database, the WHO mortality database, and the cancer incidence in five continents database (CI5plus; for adult data [age 20-74 years]). We analysed temporal trends in incidence rates, including absolute changes in rates, and the strength of the correlation between incidence rates in children and adolescents and in adults. We calculated the average annual number of thyroid cancer deaths and the age-standardised mortality rates for children and adolescents. FINDINGS: Age-standardised incidence rates of thyroid cancer among children and adolescents aged 0-19 years ranged from 0·4 (in Uganda and Kenya) to 13·4 (in Belarus) cancers per 1 million person-years in 2008-12. The variability in the incidence rates was mostly accounted for by the papillary tumour subtype. Incidence rates were almost always higher in girls than in boys and increased with age in both sexes. Rapid increases in incidence between 1998-2002 and 2008-12 were observed in almost all countries. Country-specific incidence rates in children and adolescents were strongly correlated (r>0·8) with rates in adults, as were the temporal changes in the respective incidence rates (r>0·6). Thyroid cancer deaths in those aged younger than 20 years were less than 0·1 per 10 million person-years in each country. INTERPRETATION: The pattern of thyroid cancer incidence in children and adolescents mirrors the pattern seen in adults, suggesting a major role for overdiagnosis, which, in turn, can lead to overtreatment, lifelong medical care, and side effects that can negatively affect quality of life. We suggest that the existing recommendation against screening for thyroid cancer in the asymptomatic adult population who are free from specific risk factors should be extended to explicitly recommend against screening for thyroid cancer in similar populations of children and adolescents. FUNDING: International Agency for Research on Cancer and the Union for International Cancer Control; French Institut National du Cancer; Italian Association of Cancer Research; and Italian Ministry of Health.

Kaposi sarcoma incidence, survival and trends: Data from the information network on rare cancers in Europe (RARECAREnet).

BACKGROUND: This study provides updated information on Kaposi sarcoma (KS) in Europe during 1995-2007 from the RARECARENet project. METHODS: Data comes from 59 population-based cancer registries in 22 countries. KS was defined as ICD-O-3 morphology code 9140 combined with any topography code. Crude and age-adjusted incidence rates and relative survival for years of diagnosis 2000-2007 and with trends during 1995-2007 were calculated overall, by age and by country. RESULTS: The crude annual incidence rate was 0.28 per 100,000 and age-adjusted incidence was 0.23 per 100,000; incidence increased with age, from 0.18/100,000 at age 0-44 to 0.25/100,000 at age 45-64 and 0.69/100,000 at age 65 and over. Age-adjusted incidence in males was more than four times that in females. Portugal, which had the highest incidence of AIDS in Europe, had by far the highest incidence of KS at age 0-44, 1.44/100,000, more than four times the rate in any other country. Incidence among males in Europe aged 0-44 fell significantly between 1995-1998 and 1999-2002, followed by a significant increase in 2003-2007. Younger patients, with predominantly AIDS-related KS, formerly had a worse prognosis, but since 1999-2001 5-year relative survival increased for patients aged under 65, and by 2005-2007 was 83-86 % for all three age groups 0-44, 45-64, and 65 and over. CONCLUSION: Survival and quality of life for the increasing number of people in Europe affected by KS should improve further following the development of evidence-based guidelines for its management. Population-based cancer registries will continue to play a vital role in monitoring the burden of KS and improvements in its outcome.

Severity of COVID-19 in children with cancer: Report from the United Kingdom Paediatric Coronavirus Cancer Monitoring Project.

BACKGROUND: Children with cancer are frequently immunocompromised. While children are generally thought to be at less risk of severe SARS-CoV-2 infection than adults, comprehensive population-based evidence for the risk in children with cancer is unavailable. We aimed to produce evidence of the incidence and outcomes from SARS-CoV-2 in children with cancer attending all hospitals treating this population across the UK. METHODS: Retrospective and prospective observational study of all children in the UK under 16 diagnosed with cancer through data collection from all hospitals providing cancer care to this population. Eligible patients tested positive for SARS-CoV-2 on reverse transcription polymerase chain reaction (RT-PCR). The primary end-point was death, discharge or end of active care for COVID-19 for those remaining in hospital. RESULTS: Between 12 March 2020 and 31 July 2020, 54 cases were identified: 15 (28%) were asymptomatic, 34 (63%) had mild infections and 5 (10%) moderate, severe or critical infections. No patients died and only three patients required intensive care support due to COVID-19. Estimated incidence of hospital identified SARS-CoV-2 infection in children with cancer under 16 was 3%. CONCLUSIONS: Children with cancer with SARS-CoV-2 infection do not appear at increased risk of severe infection compared to the general paediatric population. This is reassuring and supports the continued delivery of standard treatment.

Incidence of childhood renal tumours: An international population-based study.

Malignant renal tumours represent 5% of childhood cancers and include types with likely different aetiology: Wilms tumour (WT), rhabdoid renal tumour, kidney sarcomas and renal carcinomas. WT is the most common renal tumour in children, previously shown to vary internationally and with ethnicity. Using the comprehensive database of the International Incidence of Childhood Cancer study (IICC), we analysed global variations and time trends in incidence of renal tumour types in children (age 0-14 years) and adolescents (age 15-19 years). The results were presented by 14 world regions, and five ethnic groups in the US. We included 15 320 renal tumours in children and 800 in adolescents reported to the 163 contributing registries during 2001-2010. In children, age-standardised incidence rate (ASR) of renal tumours was 8.3 per million (95% confidence interval, CI = 8.1, 8.4); it was the highest in North America and Europe (9-10 per million) and the lowest in most Asian regions (4-5 per million). In the US, Blacks had the highest ASR (10.9 per million, 95% CI = 10.2, 11.6) and Asian and Pacific Islanders the lowest (4.4 per million, 95% CI = 3.6, 5.1). In adolescents, age-specific incidence rate of renal tumours was 1.4 per million (95% CI = 1.3, 1.5). WT accounted for over 90% of all renal tumours in each age from 1 to 7 years and the proportion of renal carcinomas increased gradually with age. From 1996 to 2010, incidence remained mostly stable for WT (average annual percent change, AAPC = 0.1) and increased for renal carcinomas in children (AAPC = 3.7) and adolescents (AAPC = 3.2). Our findings warrant further monitoring.

Case-control study of paternal occupational exposures and childhood bone tumours and soft-tissue sarcomas in Great Britain, 1962-2010.

BACKGROUND: This nationwide study investigated associations between paternal occupational exposure and childhood bone tumours and soft- tissue sarcomas. METHODS: The UK National Registry of Childhood Tumours provided cases of childhood sarcomas born and diagnosed in Great Britain, 1962-2010. Control births, unaffected by childhood cancer, were matched on sex, birth period and birth registration sub-district. Fathers' occupations were assigned to one or more of 33 exposure groups and coded for occupational social class. RESULTS: We analysed 5,369 childhood sarcoma cases and 5380 controls. Total bone tumours, total soft-tissue sarcomas and the subgroups osteosarcoma, rhabdomyosarcoma and Ewing Sarcoma Family of Tumours (ESFT) were considered separately. Significant positive associations were seen between rhabdomyosarcoma and paternal exposure to EMFs (odds ratio = 1.67, CI = 1.22-2.28) and also for ESFT and textile dust (1.93, 1.01-3.63). There were putative protective effects on total bone tumours of paternal dermal exposure to hydrocarbons, metal, metal working or oil mists. CONCLUSIONS: Despite the large size and freedom from bias of this study, our results should be interpreted with caution. Many significance tests were undertaken, and chance findings are to be expected. Nevertheless, our finding of associations between ESFT and paternal exposure to textile dust may support related suggestions in the literature.

Case-control study of paternal occupational exposures and childhood lymphoma in Great Britain, 1962-2010.

BACKGROUND: This nationwide study investigates associations between paternal occupational exposure and childhood lymphoma. METHODS: The UK National Registry of Childhood Tumours provided cases of childhood lymphoma born and diagnosed in Great Britain 1962-2010. Control births, unaffected by childhood cancer, were matched on sex, birth period and birth registration sub-district. Fathers' occupations were assigned to one or more of 33 exposure groups and also coded for occupational social class. RESULTS: We analysed 5033 childhood lymphoma cases and 4990 controls. Total lymphoma and the subgroups Hodgkin, Burkitt and non-Hodgkin lymphoma were considered separately. No one exposure was significantly associated with increased risk within all subgroups and for total lymphoma. However, exposure to "ceramics and glass" was significantly associated with increased risk of total lymphoma, Hodgkin and non-Hodgkin lymphoma. Paternal lead exposure was associated with Burkitt lymphoma and exposure to metal fumes was associated with Hodgkin lymphoma. CONCLUSIONS: This study provides no support for previous suggestions of an association between childhood lymphoma and paternal occupational exposure to pesticides, solvents/hydrocarbons or infections potentially transmitted by father's social contacts. An association with exposure to "ceramics and glass" was noted for the two major lymphoma subtypes together comprising 80% of total lymphoma.

Incidence of childhood CNS tumours in Britain and variation in rates by definition of malignant behaviour: population-based study.

BACKGROUND: Intracranial and intraspinal tumours are the most numerous solid tumours in children. Some recently defined subtypes are relatively frequent in childhood. Many cancer registries routinely ascertain CNS tumours of all behaviours, while others only cover malignant neoplasms. Some behaviour codes have changed between revisions of the International Classification of Diseases for Oncology, including pilocytic astrocytoma, downgraded to uncertain behaviour in ICD-O-3. METHODS: We used data from the population-based National Registry of Childhood Tumours, which routinely included non-malignant CNS tumours, to document the occurrence of CNS tumours among children aged < 15 years in Great Britain during 2001-2010 and to document the descriptive epidemiology of childhood CNS tumours over the 40-year period 1971-2010, during which several new entities were accommodated in successive editions of the WHO Classification and revisions of ICD-O. Eligible cases were all those with a diagnosis included in Groups III (CNS tumours) and Xa (CNS germ-cell tumours) of the International Classification of Childhood Cancer, Third Edition. The population at risk was derived from annual mid-year estimates by sex and single year of age compiled by the Office for National Statistics and its predecessors. Incidence rates were calculated for age groups 0, 1-4, 5-9 and 10-14 years, and age-standardised rates were calculated using the weights of the world standard population. RESULTS: Age-standardised incidence in 2001-10 was 40.1 per million. Astrocytomas accounted for 41%, embryonal tumours for 17%, other gliomas for 10%, ependymomas for 7%, rarer subtypes for 20% and unspecified tumours for 5%. Incidence of tumours classified as malignant and non-malignant by ICD-O-3 increased by 30 and 137% respectively between 1971-75 and 2006-10. CONCLUSIONS: Total incidence was similar to that in other large western countries. Deficits of some, predominantly low-grade, tumours or differences in their age distribution compared with the United States and Nordic countries are compatible with delayed diagnosis. Complete registration regardless of tumour behaviour is essential for assessing burden of disease and changes over time. This is particularly important for pilocytic astrocytoma, because of its recent downgrading to non-malignant and time trends in the proportion of astrocytomas with specified subtype.

Survival of adults with cancers of bone or soft tissue in Europe-Report from the EUROCARE-5 study.

BACKGROUND: Five-year relative survival (RS) of adults with bone and soft-tissue cancers in Europe was still <60% by 1995-1999. There was large geographical survival variability, mainly for bone tumours, and survival decreased with increasing age at diagnosis. METHODS: Data from 87 population-based cancer registries in 29 countries, extracted from the EUROCARE-5 database, were used to provide updated estimates of survival and describe trends in survival of adults with cancers of these sites across Europe. We calculated 5-year RS for patients diagnosed in 2000-2007. We estimated 5-year RS by the period approach to assess changes in survival between 1999-2001, 2002-2004 and 2005-2007, and provide reliable predictions for recently diagnosed patients. RESULTS: Five-year RS was 60% for adults diagnosed with soft-tissue cancer in 2000-2007 and 53% for those with bone cancer. RS declined with increasing age at diagnosis, especially for bone cancer. Survival from bone cancer varied widely between European regions, from 63 to 62% in Northern and Central Europe to 39% in Eastern Europe. Inter-regional variation was much less for soft-tissue cancer. For both site groupings, there was little evidence of change in five-year RS up to 2002-2004, followed by increases of 3-4% during 2005-2007. CONCLUSIONS: Outcomes for adults with bone and soft-tissue cancer in Europe began to improve around 2005; new therapeutic developments are expected to result in further progress. Survival improvements already achieved must be brought more fully to elderly patients and those in Eastern Europe. European Reference Networks on rare cancers will have a vital role in future progress.