Charles A Stiller

• Lead on Childhood Cancer at Public Health England

Background International variation in childhood cancer survival might be explained by differences in stage at diagnosis, among other factors. As part of the BENCHISTA project, we aimed to assess geographical variation in tumour stage at diagnosis through the application, by population-based cancer registries working with clinicians, of the internat...

Importance Studies on the epidemiology of retinoblastoma (RB) could lead to improvement in management. Objective To estimate the incidence and survival of RB in European children and the occurrence of second primary tumors (other than RB) in these patients. Design, Setting, and Participants This cohort study used population-based data from 81 can...

Childhood cancer survivors (CCS) require specialized follow-up throughout their lifespan to prevent or manage late effects of cancer treatment. Knowing the size and structure of the population of CCS is crucial to plan interventions. In this scoping review we reviewed studies that reported prevalence of CCS in Europe. We searched Medline, Web of Sc...

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...

Background: We used the comprehensive definition of AYA (age 15 to 39 years) to update 5-year relative survival (RS) estimates for AYAs in Europe and across countries and to evaluate improvements in survival over time. Methods: We used data from EUROCARE-6. We analysed 700,000 AYAs with cancer diagnosed in 2000-2013 (follow-up to 2014). We focused...

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...

Introduction Variation in stage at diagnosis of childhood cancers (CC) may explain differences in survival rates observed across geographical regions. The BENCHISTA project aims to understand these differences and to encourage the application of the Toronto Staging Guidelines (TG) by Population-Based Cancer Registries (PBCRs) to the most common sol...

Background Establishing the existence of health inequalities remains a high research and policy agenda item in the United Kingdom. We describe ethnic and socio-economic differences in paediatric cancer survival, focusing specifically on the extent to which disparities have changed over a 20-year period. Methods Cancer registration data for 2674 ch...

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 Brit...

Background Survival is a key metric of the effectiveness of a health system in managing cancer. We set out to provide a comprehensive examination of worldwide variation and trends in survival from brain tumors in adults, by histology. Methods We analyzed individual data for adults (15–99 years) diagnosed with a brain tumor (ICD-O-3 topography code...

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...

Introduction: Tumours of the central nervous system are among the leading causes of cancer-related death in children. Population-based cancer survival reflects the overall effectiveness of a health care system in managing cancer. Inequity in access to care world-wide may result in survival disparities. Methods: We considered children (0-14 years...

Background Ductal adenocarcinomas and neuroendocrine tumours are the two main morphological sub-types of pancreatic cancer. Using data from CONCORD-3, we examined whether the distribution of morphological sub-types could help explain international variations in pancreatic cancer survival for all morphologies combined. We also examined world-wide su...

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 D...

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 yo...

Background: CONCORD-3 highlighted wide disparities in population-based 5-year net survival during 2000-2014. Clinical evidence suggests marked international differences in the proportion of lethal acral and nodular subtypes. Objectives: We aim to assess whether the differences in morphology may explain global variation in survival. Methods: We...

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 Cor...

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 cou...

Background: Oral etoposide is commonly used in palliative treatment of childhood and young adult cancer without robust evidence. We describe a national, unselected cohort of young people in England treated with oral etoposide using routinely collected, population-level data. Methods: Patients aged under 25 years at cancer diagnosis (1995-2017) w...

Introduction: Global variations in survival for brain tumours 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. Patients and methods: The source for the analysis was the CONCORD data ba...

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 survi...

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. Me...

Cancer types in adolescents and young adults form a unique distribution. A system for classifying them is presented.

Under 2% of all cancers in industrialized countries occur in childhood and adolescence, but they account for a much larger proportion of total population life years potentially lost to cancer. Total incidence is about 160 per million in children and 200 per million in adolescents. In children, leukaemias account for one third of all malignancies an...

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 Internationa...

This nationwide study investigated associations between paternal occupational exposure and childhood bone tumours and soft- tissue sarcomas. 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 per...

This nationwide study investigates associations between paternal occupational exposure and childhood lymphoma. 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-...

Abstract 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 t...

Medulloblastoma can occur at any age, but is most frequent in infancy and childhood. There have been no previous detailed reports of incidence or survival across all age groups in the UK and few population-based reports internationally. This project provides for the first time population-based data on medulloblastoma incidence and survival across a...

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 fr...

Background: Significant research on the epidemiology and natural history of childhood cancer took place in the Universities of Oxford and Birmingham over sixty years. This is the first of three papers recording this work and describes the Oxford Survey of Childhood Cancers (OSCC), the largest case-control survey of childhood cancer ever undertaken...

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 inv...

Background: High doses of ionising radiation are a known cause of childhood cancer and great public and professional interest attaches to possible links between childhood cancer and lower doses, particularly of man-made radiation. This paper describes work done by the Childhood Cancer Research Group (CCRG) on this topic METHODS: Most UK investigat...

Background: We summarise the work of the Childhood Cancer Research Group, particularly in relation to the UK National Registry of Childhood Tumours (NRCT). Methods: The Group was responsible for setting up and maintaining the NRCT. This registry was based on notifications from regional cancer registries, specialist children's tumour registries,...

Neoplasms are rare in neonates, although somewhat more frequent than in older children. In Great Britain, for birth years 1988–2007, the National Registry of Childhood Tumours recorded 394 cases of cancer (including non-malignant CNS tumours) in live-born infants less than 28 days of age. The risk of neonatal cancer was 27.6 per million live births...

Background: Studies have shown marked improvements in survival between 1981 and 2000 for Ewing sarcoma patients but not for osteosarcoma. This study aimed to explore socio-economic patterning in early mortality rates for both tumours. Procedure: The study analysed all 2432 osteosarcoma and 1619 Ewing sarcoma cases, aged 0-49 years, diagnosed in Gre...

In 2015, the second cycle of the CONCORD programme established global surveillance of cancer survival as a metric of the effectiveness of health systems and to inform global policy on cancer control. CONCORD-3 updates the worldwide surveillance of cancer survival to 2014. Methods CONCORD-3 includes individual records for 37·5 million patients diagn...

Background: Studies have shown marked improvements in survival between 1981 and 2000 for Ewing sarcoma patients but not for osteosarcoma. This study aimed to explore socio-economic patterning in early mortality rates for both tumours. Procedure: The study analysed all 2432 osteosarcoma and 1619 Ewing sarcoma cases, aged 0-49 years, diagnosed in...

Background: In 2015, the second cycle of the CONCORD programme established global surveillance of cancer survival as a metric of the effectiveness of health systems and to inform global policy on cancer control. CONCORD-3 updates the worldwide surveillance of cancer survival to 2014. Methods: CONCORD-3 includes individual records for 37·5 millio...

This study aimed to compare cancer incidence and trends in survival for children diagnosed in Japan and England, using population-based cancer registry data. The analysis was based on 5,192 children with cancer (age 0-14 years) from six prefectural cancer registries in Japan and 21,295 children diagnosed in England during 1993-2010. Differences in...

Study question: Do children born after donor ART have an increased risk of developing childhood cancer in comparison to the general population? Summary answer: This study showed no overall increased risk of childhood cancer in individuals born after donor ART. What is known already: Most large population-based studies have shown no increase in...

CNS tumors are least likely to present during adolescence, when they do, their clinical presentation is enmeshed with the young person’s physical, psychological, educational, and social development. The population incidence of the different tumor types highlights the transition from “childhood type” to “adult type” and identifies those driven by ad...

Objective Participation rates in clinical trials are low in teenagers and young adults (TYA) with cancer. Whilst the importance of clinical trials in informing best practice is well established, data regarding individual patient benefit are scarce. We have investigated the association between overall survival and trial recruitment in TYA patients w...

Survival for childhood central nervous system (CNS) tumours varies across Europe, partly because of the difficulty of distinguishing malignant from non-malignant disease. This study examines bias in CNS tumours survival analysis to obtain the reliable and comparable survival figures. We analysed survival data for about 15,000 children (age <15) dia...

Background Rare cancers pose challenges for diagnosis, treatments, and clinical decision making. Information about rare cancers is scant. The RARECARE project defined rare cancers as those with an annual incidence of less than six per 100 000 people in European Union (EU). We updated the estimates of the burden of rare cancers in Europe, their time...

Aims Cancer incidence has been investigated in children born after non-donor assisted conception, but incidence in children born after donor assisted conception remain uncertain. This study aimed to determine overall and site specific cancer incidence in a British cohort of children born after assisted conception using donor gametes. Methods This...

Background Cancer is a major cause of death in children worldwide, and the recorded incidence tends to increase with time. Internationally comparable data on childhood cancer incidence in the past two decades are scarce. This study aimed to provide internationally comparable local data on the incidence of childhood cancer to promote research of cau...

Background: Global inequalities in access to health care are reflected in differences in cancer survival. The CONCORD programme was designed to assess worldwide differences and trends in population-based cancer survival. In this population-based study, we aimed to estimate survival inequalities globally for several subtypes of childhood leukaemia....

Background Cancer is a major cause of death in children worldwide, and the recorded incidence tends to increase with time. Internationally comparable data on childhood cancer incidence in the past two decades are scarce. This study aimed to provide internationally comparable local data on the incidence of childhood cancer to promote research of cau...

Background The role of age as a prognostic factor has been examined in single institutional studies and in larger data sets from the SEER database, showing a survival advantage for younger versus adult patients with synovial sarcoma (SS). To further assess the role of age, socioeconomic status and other prognostic factors on outcome for SS, we anal...

Background Efforts to improve end of life care (EoLC) have made tangible impacts on care in adults, including enabling more people to die at their preferred place of death (PoD), usually home or hospices. Little is known how the PoD in children and young people (CYP, ≤24 years) has changed over time, especially in the context of a series of nationa...

Background: Data from EUROCARE have consistently shown lower survival for adolescents and young adults (AYAs; aged 15-24 years) than for children (0-14 years) for most cancers that affect both groups, and modest survival improvements up to 2000-02. AYAs have longer survival than that of adults for most cancers. We used the latest definition of AYA...

Cancer typically has an annual incidence of 150 per million children under 15 years of age in industrialised countries. Leukemia accounts for about one third of total incidence and CNS tumors (including non-malignant) for one quarter. Lymphomas, soft-tissue sarcomas, neuroblastoma and renal tumours each account for 5–10 %. Most cancers of most site...

Objectives To examine the trend and geographical variation in place of death (PoD) for children and young people (CYP) who died with cancer and factors associated with these variations, in the context of a series of national initiatives for end of life care (EoLC) improvement in adults since late 1990s. Design and setting Population-based cross-sec...

Background: We previously reported evidence of a dose–response relationship between ionising-radiation exposure from paediatric computed tomography (CT) scans and the risk of leukaemia and brain tumours in a large UK cohort. Underlying unreported conditions could have introduced bias into these findings. Methods: We collected and reviewed additiona...

Cancers in children under 15 years of age are histologically diverse and include entities hardly ever found in older people. Annual incidence is usually between 70 and 160 per million. The most frequent types are leukemias, Central nervous system tumors, lymphomas, sarcomas, and characteristic embryonal tumors of childhood. The etiology of most chi...

Wilms tumor is the most common childhood renal cancer. To identify mutations that predispose to Wilms tumor, we are conducting exome sequencing studies. Here we describe 11 different inactivating mutations in the REST gene (encoding RE1-silencing transcription factor) in four familial Wilms tumor pedigrees and nine non-familial cases. Notably, no s...

Although relatively rare, cancer in teenagers and young adults (TYA) is the most common disease-related cause of death and makes a major contribution to years of life lost in this age group. There is a growing awareness of the distinctive needs of this age group and drive for greater understanding of how outcomes can be improved. We present here th...

We aimed to describe and compare survival in teenagers and young adults (TYAs) with cancer to that of younger children and older adults, to identify sub-populations at greater or lesser risk of death. We compared survival in cancer patients diagnosed in the United Kingdom aged 13-24years (TYAs) to those aged 0-12 (children) and 25-49years (adults)...

Although relatively rare, cancer in teenagers and young adults (TYA) is the most common disease-related cause of death and makes a major contribution to years of life lost in this age group. There is a growing awareness of the distinctive needs of this age group and drive for greater understanding of how outcomes can be improved. We present here th...

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...

Worldwide data for cancer survival are scarce. We aimed to initiate worldwide surveillance of cancer survival by central analysis of population-based registry data, as a metric of the effectiveness of health systems, and to inform global policy on cancer control. Individual tumour records were submitted by 279 population-based cancer registries in...

Kaposi sarcoma (KS) is a virus-related malignancy which most frequently arises in skin, though visceral sites can also be involved. Infection with Kaposi sarcoma herpes virus (KSHV or HHV-8) is required for development of KS. Nowadays, most cases worldwide occur in persons who are immunosuppressed, usually because of HIV infection or as a result of...

Primary renal tumours, predominantly Wilms tumour, account for 4–7 % of all childhood cancers. The incidence of renal cancer among children and adolescents in industrialised countries is presented in detail. Worldwide variations in incidence, which are predominantly on ethnic rather than geographical lines, are discussed. The available information...

Wilms tumour is a childhood kidney cancer. Here we identify inactivating CTR9 mutations in 3 of 35 Wilms tumour families, through exome and Sanger sequencing. By contrast, no similar mutations are present in 1,000 population controls (P<0.0001). Each mutation segregates with Wilms tumour in the family and a second mutational event is present in ava...

BACKGROUND: In recurrent neuroectodermal brain tumors leptomeningeal disease manifestation was observed in about 50 to 75% of patients. The role and efficacy of intrathecal therapy in these patients is unclear. As the systemic administration of etoposide is efficacious in brain tumors a phase II study was initiated to evaluate the efficacy and safe...

We report a population-based study examining long-term outcomes for common pediatric CNS tumors comparing results from the UK with the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) data set and with the literature. No such international study has previously been reported. Data between 1996 and 2005 from the UK Natio...

To assess recruitment of children to national clinical trials for acute lymphoblastic leukaemia (ALL) and acute myeloid leukaemia (AML) in Great Britain during 1980-2007 and describe variation by some factors that might influence trial entry. Records of leukaemia patients aged 0-14 years at diagnosis were identified in the National Registry of Chil...

Artificial fluoridation of drinking water to improve dental health has long been a topic of controversy. Opponents of this public health measure have cited the possibility of bone cancer induction. The study objective was to examine whether increased risk of primary bone cancer was associated with living in areas with higher concentrations of fluor...

Previously we identified space-time clustering in certain childhood cancers. The present study aimed to determine whether there was cross-space-time clustering between different diagnostic groups. A total of 32,295 cases were diagnosed during 1969-1993. Cross-space-time clustering was analysed by a second-order procedure based on Diggle's method. L...

Summary Background 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...

Accurate population-based data are needed on the incidence of cancer in children born after assisted conception. We linked data on all children born in Britain between 1992 and 2008 after assisted conception without donor involvement with data from the United Kingdom National Registry of Childhood Tumours to determine the number of children in whom...

Background: Concern about the risk of leukaemia in children living near nuclear power plants (NPPs) persists. Previous British analyses have been area based and consequently thought to be less effective than case–control studies. Methods: Cases of childhood leukaemia and non-Hodgkin lymphoma (LNHL) born and diagnosed in Great Britain between 1962...