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Biomedical subjects

C A Stiller

Publications and source records attributed to C A Stiller.

At least 19 recordsLinked to original sources
PubMed2006

Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour.

Wilms tumour has been reported in association with over 50 different clinical conditions and several abnormal constitutional karyotypes. Conclusive evidence of an increased risk of Wilms tumour exists for only a minority of these conditions, including WT1 associated syndromes, familial Wilms tumour, and certain overgrowth conditions such as Beckwith-Wiedemann syndrome. In many reported conditions the rare co-occurrence of Wilms tumour is probably due to chance. However, for several conditions the available evidence cannot either confirm or exclude an increased risk, usually because of the rarity of the syndrome. In addition, emerging evidence suggests that an increased risk of Wilms tumour occurs only in a subset of individuals for some syndromes. The complex clinical and molecular heterogeneity of disorders associated with Wilms tumour, together with the apparent absence of functional links between most of the known predisposition genes, suggests that abrogation of a variety of pathways can promote Wilms tumorigenesis.

Chromosome Aberrations↗
PubMed2006

Patterns of care and survival for patients aged under 40 years with bone sarcoma in Britain, 1980-1994.

The purpose of the study was to calculate population-based survival rates for osteosarcoma (OS) and Ewing's sarcoma (ES) in Great Britain during 1980-1994, determine proportions of patients treated at specialist centres or entered in national and international clinical trials, and investigate effects of these factors on survival. Data on a population-based series of 1349 patients with OS and 849 with ES were compiled from regional and national cancer registries, UK Children's Cancer Study Group, regional bone tumour registries and clinical trials. Follow-up was through population registers. Survival was analysed by actuarial analysis with log-rank tests and by Cox's proportional hazards analysis. Five-year survival rates during 1980-1984, 1985-1989 and 1990-1994 were 42% (95% CI: 37, 46), 54% (95% CI: 50, 59) and 53% (95% CI: 48, 57), respectively, for OS and 31% (95% CI: 26, 37), 46% (95% CI: 40, 51) and 51% (95% CI: 45, 57) for ES. Proportions of patients treated at a supraregional bone tumour centre or a paediatric oncology centre in the three quinquennia were 36, 56 and 67% for OS and 41, 60 and 69% for ES. In 1983-1992, 48% of OS patients were entered in a national trial; for ES, 27% were entered in 1980-1986 and 54% in 1987-1994. Survival was similar for trial and nontrial patients with OS. For ES, trial patients had consistently higher 5-year survival than nontrial patients: 1980-1986, 42 vs 30%; 1987-1992, 59 vs 42%; 1993-1994, 54 vs 43%. During 1985-1994, patients with OS or ES whose main treatment centre was a nonteaching hospital had lower survival rates. In multivariate analyses of patients diagnosed during 1985-1994 that also included age, sex, primary site, surgical treatment centre, the results relating to main treatment centre for both OS and ES retained significance but the survival advantage of trial entry for ES became nonsignificant. For both OS and ES diagnosed since 1985, patients whose main treatment centre was a nonspecialist hospital had a lower survival rate.

Adolescent↗
PubMed2006

Liver cancer in European children: incidence and survival, 1978-1997. Report from the Automated Childhood Cancer Information System project.

Data on 849 children diagnosed with malignant hepatic tumours (International Classification of Childhood Cancer, Group VII) before the age of 15 years during 1978-1997 in Europe were extracted from the ACCIS database. Age-standardised incidence during 1988-1997 was 1.5 per million overall, 1.2 per million for hepatoblastoma and 0.2 per million for hepatic carcinoma. Over 90% of cases of hepatoblastoma occurred before age 5 years, whereas hepatic carcinoma had a fairly flat age distribution. Both tumours had an incidence in boys of 1.5-1.6 times that in girls. There were no significant time trends in incidence during 1978-1997. Five-year survival from hepatoblastoma diagnosed during 1988-1997 was 63% overall, and ranged from 52% in Eastern Europe to 84% in the North. Survival from hepatic carcinoma was much lower (37%). Between 1978-1982 and 1993-1997, 5-year survival (95% confidence interval (95% CI)) increased from 28% (95% CI 18-39) to 66% (95% CI 55-74) for hepatoblastoma and from 17% (95% CI 6-33) to 50% (95% CI 26-70) for hepatic carcinoma. These increases reflect the impact of advances in treatment of childhood liver cancer at a population level.

Adolescent↗
PubMed2006

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.

Adolescent↗
PubMed2006

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.

Adolescent↗
PubMed2006

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.

Adolescent↗
PubMed2006

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.

Adolescent↗
PubMed2006

Cancer in children and adolescents in Europe: developments over 20 years and future challenges.

This special issue contains 18 articles describing population-based analyses of incidence and survival for cancer among children and adolescents in Europe over the period 1978-1997. The analyses were derived from the large database of the ACCIS project (Automated Childhood Cancer Information System), which was built through collaboration of 62 population-based cancer registries in 19 European countries. Data on 88,465 cancers in children and 15,369 in adolescents (age 15-19 yrs) were included in the various analyses, making this the largest database on cancer in these age-groups in the world. National data were grouped into five European regions to allow comparisons of incidence and survival, for all cancers and by tumour type, including analysis of trends in both over time. This overview paper focuses on the comparability of the data from multiple registries and describes the potential confounding factors. Age-standardised annual incidence rates of many, but not all, cancers in children and adolescents are clearly rising. There are geographical differences in survival for the majority of tumour types. Survival rates increased for nearly all types of cancer in children and adolescents. The implications of these findings for aetiological factors and treatment delivery for cancer in children and adolescents are discussed.

Adolescent↗
PubMed2004

Long-term population-based risks of second malignant neoplasms after childhood cancer in Britain.

In a population-based, retrospective cohort study of 16 541 3-year survivors of childhood cancer treated in Britain up to the end of 1987, 278 second malignant neoplasms (SMNs) were identified against 39.4 expected giving a standardised incidence ratio (SIR) of 6.2. The overall cumulative risk of an SMN by 25 years from 3-year survival from childhood cancer was 4.2%. Analysis of the cohort of nonretinoblastoma childhood cancers combined revealed a significant decline in SIR of SMN with increasing duration of follow-up. There was a greater risk of developing a SMN, particularly secondary acute myeloid leukaemia, in those diagnosed with childhood cancer from 1980 onwards. However, on multivariate modeling, this was not an independent risk factor. There was significant heterogeneity (P<0.001) in SIR of SMN across different treatment groups, the greatest risk observed in the group exposed to both radiotherapy and chemotherapy. The risks of SMN observed were comparable with those in other population-based studies. While the decline in SIR with duration of follow-up and the small excess numbers of cancers observed over later decades after diagnosis are reassuring, the high excess risk, particularly of leukaemia, associated with recent more intense therapy is of concern.

Acute Disease↗
PubMed2001

Season of birth and diagnosis of children with leukaemia: an analysis of over 15 000 UK cases occurring from 1953-95.

If infections are involved in the aetiology of childhood leukaemia then seasonal variation in the birth or onset dates of the malignancy may be apparent. Previous studies that have examined seasonality of these dates have produced conflicting results. Using population-based data from the National Registry of Childhood Tumours we conducted a larger study than any to date of 15 835 cases of childhood leukaemia born and diagnosed in the UK between 1953-95. We found no evidence of seasonality in either month of birth or month of diagnosis overall or in any subgroups by age, sex, histology or immunophenotype. We did however find a significant (P = 0.01) February peak in month of birth for cases born before 1960 and a significant (P = 0.02) August peak in month of diagnosis for those diagnosed before 1962. Whilst these findings may be due to chance they are also consistent with changes over time in the seasonality of exposure, or immunological response, to a relevant infection. Changes in the seasonal variation in the fatality rate of a pre-leukaemic illness, such as pneumonia, could be another explanation.

Adolescent↗
PubMed2001

Childhood cancer and parental use of tobacco: findings from the inter-regional epidemiological study of childhood cancer (IRESCC).

Parental smoking data have been re-abstracted from the interview records of the Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC) to test further the hypothesis that paternal cigarette smoking is a risk factor for the generality of childhood cancer. Reported cigarette smoking habits for the parents of 555 children diagnosed with cancer in the period 1980-1983 were compared, in two separate matched pairs analyses, with similar information for the parents of 555 children selected from GP lists (GP controls) and for the parents of 555 hospitalized children (hospital controls). When cases were compared with GP controls there was a statistically significant positive trend (P = 0.02) between the risk of childhood cancer and paternal daily consumption of cigarettes before the pregnancy; there was no significant trend for maternal smoking habit. When cases were compared with hospital controls there was a statistically significant negative trend (P< 0.001) between the risk of childhood cancer and maternal daily consumption of cigarettes before the pregnancy; there was no significant trend for paternal smoking habit. Neither of the significant trends could be explained by adjustment for socioeconomic grouping, ethnic origin or parental age at the birth of the child, or by simultaneous analysis of parental smoking habits. Relations between maternal consumption of cigarettes and birth weights suggested that (maternal) smoking data were equally reliable for case and control subjects, although comparisons with national data suggested that the hospital control parents were unusually heavy smokers. These findings give some support for the hypothesis that paternal cigarette smoking is a potential risk factor for the generality of childhood cancers.

Adult↗
PubMed2001

Survival of children with bone sarcoma in Europe since 1978: results from the EUROCARE study.

Malignant bone tumours in children are rare, accounting for approximately 5% of all childhood cancers in European countries. In the EUROCARE childhood cancer study, there were 1785 registrations from 16 countries for bone cancers in patients aged 0--14 years during 1978--1989. Of this total, almost three-quarters were contributed by childhood cancer registries in Germany and the UK. Estimated 5-year survival rates were 52% for osteosarcoma and 50% for Ewing's sarcoma over the entire study period and 60% for both diagnostic groups in 1985--1989. For osteosarcoma, survival rates increased substantially until about 1985, but then showed no further improvement. For Ewing's sarcoma, there was a steady increase throughout the study period. Improvements in survival which had previously been reported from individual countries and in clinical series are confirmed as having taken place throughout much of Europe on a population basis.

Adolescent↗
PubMed2001

Survival of children with soft-tissue sarcoma in Europe since 1978: results from the EUROCARE study.

Soft-tissue sarcomas account for 5--8% of all childhood cancers in European countries. In the EUROCARE childhood cancer study, there were 2163 registrations from 17 countries for soft-tissue sarcomas in patients aged 0--14 years during 1978--1989. Of this total, three-quarters were contributed by childhood cancer registries in Germany and the UK. Age-standardised 5-year survival rates of children diagnosed during 1985--1989 were 65% (95% confidence interval (CI) 56--72) for rhabdomyosarcoma, 68% (95% CI 58--77) for fibrosarcoma, 78% (95% CI: 64--87) for other specified soft-tissue sarcomas except Kaposi's and 51% (95% CI 37--65) for 'unspecified' soft-tissue sarcomas. Survival rates increased steadily throughout the 12-year study period for all soft-tissue sarcomas combined, but the increase took place predominantly in the early 1980s for rhabdomyosarcoma. Improvements in survival which had previously been reported from individual countries and in clinical series are confirmed as having taken place throughout much of Europe on a population basis. In a supplementary analysis, there was little indication of a further improvement during 1990--1992.

Adolescent↗
PubMed2001

Survival of children with Wilms' tumour in Europe.

A total 2535 cases of Wilms' tumours registered in children aged 0--14 years by 34 population-based cancer registries in 16 countries of Europe in 1978--1992 and followed-up until the end of 1994 were included in this EUROCARE study. Overall 5-year observed survival of all children diagnosed in 1985--1989 was 83%, 95% confidence interval (CI) 80--85. Relatively large differences were observed between the European countries, with significantly lower survival of patients registered in the formerly socialist countries, Estonia, Poland and Slovakia. Overall European survival was slightly lower in comparison with results reported from the USA and Australia, which demonstrate a potential for improvement. Over the study period, overall survival adjusted for age, sex and country has increased significantly. This favourable trend is attributed primarily to improvements in treatment, particularly to the introduction of new chemotherapeutic agents.

Adolescent↗
PubMed2000

Haematologists' approaches to the management of adolescents and young adults with acute leukaemia.

Approaches to the management of adolescents and young adults with acute leukaemia were investigated by sending a questionnaire to hospitals identified as having diagnosed or treated patients aged 15-29 years. The responses demonstrated the types of hospital treating these patients, the haematologists' perceived practice for entry of patients to Medical Research Council (MRC) leukaemia trials and reasons for non-entry. Data were linked to MRC trials data to determine the proportion of patients aged 15-29 years at diagnosis in responding hospitals actually treated in MRC leukaemia trials in the 5 years preceding the questionnaire. Eighty-two per cent of haematologists stated that they entered patients 'always' or 'whenever possible' for acute myeloid leukaemia (AML) and 76% for acute lymphoblastic leukaemia (ALL), but actual entry rates from the study hospitals were 46% of 239 AML patients and 36% of 182 ALL patients. The reasons most commonly reported for not entering eligible patients to national leukaemia trials were clinician preference for one arm of an MRC trial, a regional study or non-trial protocol, and concern about workload and ethical approval.

Acute Disease↗
PubMed2000

Ethnic group and survival from childhood cancer: report from the UK Children's Cancer Study Group.

Survival following cancer was analysed in relation to ethnic group among children diagnosed in Britain during 1981-1996 and treated at paediatric oncology centres by members of the UK Children's Cancer Study Group. Survival was analysed for 11 diagnostic groups: acute lymphoblastic leukaemia (ALL), acute non-lymphocytic leukaemia, Hodgkin's disease, non-Hodgkin's lymphoma, astrocytoma, primitive neuroectodermal tumour, neuroblastoma, Wilms' tumour, osteosarcoma, Ewing's sarcoma and rhabdomyosarcoma. There were no significant differences in survival between White and non-White children over the study period as a whole. Among children with ALL, however, the relative risk of death allowing for period of diagnosis, age and white blood count was 1.25 for those of South Asian ethnic origin compared with Whites (P = 0.057).

Adolescent↗