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Background: Event-free survival (EFS) considers other adverse events in addition to mortality. It therefore provides a more complete understanding of the effectiveness and consequences of treatment than standard survival measures, but is rarely reported at the population level for childhood cancer., Procedure: Our study cohort (n = 7067) was obtained from the Australian Childhood Cancer Registry, including children aged under 15 diagnosed with cancer between 2006 and 2015, with follow-up potentially available to 31 December 2020. The events of interest were relapse following remission, progressive disease, diagnosis of a second primary cancer or death from any cause. Five-year EFS and all-cause observed survival were both calculated, stratified by type of childhood cancer, remoteness of residence and stage at diagnosis. Differences in EFS were assessed using multivariable flexible parametric models., Results: Approximately one quarter of patients (n = 1605 of 7067, 23%) experienced at least one of the events of interest within 5 years of diagnosis. Relapse was twice as common for children with metastatic/advanced disease (22%) versus children with localised/limited cancers (11%). Overall 5-year EFS was 75.0% (95% confidence interval [CI]: 73.9%-76.0%), compared to 85.8% observed survival (95% CI: 85.0%-86.6%). Patients with other gliomas had the lowest EFS (35.4%, 95% CI: 27.8%-43.1%). EFS was significantly lower among children with acute myeloid leukaemia in outer regional/remote areas compared to major cities (adjusted hazard ratio [HR] = 1.90, 95% CI: 1.20-3.00)., Conclusions: Reporting EFS at a population level provides further insight on a wider range of impacts apart from mortality alone, contributing towards efforts to improve the management and outcomes of childhood cancer., (© 2024 Wiley Periodicals LLC.)

Background: An international expert panel recently recommended 15 'non-stage prognostic indicators' (NSPIs) across eight childhood cancers, classified as essential or additional, for collection in population-based cancer registries. We aimed to describe the incidence distribution and survival of each of these NSPIs., Procedures: Cases were extracted from the Australian Childhood Cancer Registry. The study cohort (n = 4187) comprised all children aged under 15 years diagnosed with an eligible cancer between 2010 and 2018, with follow-up until 31 December 2020. NSPI data were collected directly from each patient's medical records. Differences in 5-year relative survival were assessed using multivariable flexible parametric models, adjusted for sex and age group at diagnosis., Results: The availability of data varied, exceeding 85% for all essential NSPIs apart from histologic subtype for Wilms tumours (69%) and lineage for acute lymphoblastic leukaemia (78%). Information on additional NSPIs tended to be recorded less often, particularly cytogenetic subtype for non-alveolar rhabdomyosarcoma (28%) and astrocytoma (4%). Eight NSPIs exhibited a significant difference in survival, with the largest disparity occurring among children with astrocytoma according to tumour grade (5-year relative survival of 18% for grade IV disease compared with 99% for grade I disease; p < .001)., Conclusions: Our findings demonstrate that most of the recommended NSPIs can be retrieved from medical records in Australia in recent years, allowing the capability of assessing survival within patient subgroups of clinical interest. Reporting of NSPI data has the capability to inform local and global understanding of population-level disparities in childhood cancer survival., (© 2024 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Survivors of childhood cancer have an increased risk of long-term health issues arising mostly from the side effects of treatment. Using population-based data from the Australian Childhood Cancer Registry (ACCR) for children aged 0-14 at diagnosis between 1983 and 2018, there were a total of 17,468 prevalent cases of childhood cancer survivors on 31 December 2018. We also found an 80% increase in the number of 5-year prevalent cases, from 1979 in 1988 to 3566 in 2018. Both short- and long-term prevalence estimates are important for monitoring childhood cancer survivorship and planning for the specific needs of this expanding cohort., (© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Estimates of childhood cancer survival are usually reported at 5 years after diagnosis only. Using cases prevalent between 2014 and 2018 from the population-based Australian Childhood Cancer Registry, we used the period method to calculate relative survival up to 20 years post diagnosis by cancer type. Twenty-year relative survival for all childhood cancers combined (n = 14,353) was 83.8% (95% confidence interval [CI] = 82.6%-85.0%). Survival decreased only slightly after 10 years for most childhood cancers, except for some types of brain and liver tumours. These contemporary estimates of long-term survival provide valuable information to assist childhood cancer patients and their families in planning for the future., (© 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Background: This study reports cancer incidence and survival among Aboriginal and Torres Strait Islander children and other Australian children, and assesses changes over time., Procedure: Data were from the population-based Australian Childhood Cancer Registry. The study comprised children aged under 15 diagnosed between 1997 and 2016 and with mortality follow-up until 31 December 2017. Incidence trends were analysed using JoinPoint regression. Five-year cancer-specific survival was calculated using the semi-complete approach with survival comparisons made using multivariable flexible parametric models., Results: Aboriginal and Torres Strait Islander children accounted for 506 of 13,299 eligible cases (3.8%). Incidence rates for Aboriginal and Torres Strait Islander children across the study period increased by 2.3% annually (95% confidence interval [CI]: +0.6% to +4.0%) and for other Australian children increased by 0.6% annually (95% CI: +0.3% to +0.9%; p = .05). Nonetheless, cancer incidence was consistently lower for Aboriginal and Torres Strait Islander children, with an incidence rate ratio of 0.73 (95% CI: 0.62-0.85; p < .01) between 2012 and 2016. Survival for Aboriginal and Torres Strait Islander children with solid tumours was 70.6% (95% CI: 62.5%-77.3%) and for other Australian children was 83.5% (95% CI: 82.3%-84.7%; p < .01), with indications of this difference diminishing in recent years., Conclusions: Improvements in identification, particularly in urban areas, most likely accounts for the greater increase in cancer incidence rates among Aboriginal and Torres Strait Islander children. Examination of data on stage at diagnosis and treatment may provide important insights into survival for children with solid tumours., (© 2021 Commonwealth of Australia. Pediatric Blood & Cancer © 2021 Wiley Periodicals LLC.)

Background: People who receive treatment for cancer during childhood often experience subsequent complications of therapy, known as late effects, which can lead to an increased risk of death., Procedure: Using deidentified population-based data from the Australian Childhood Cancer Registry for children aged 0-14 diagnosed with cancer during the period 1983-2011 and who survived for a minimum of 5 years, we examined disease-related deaths (other than cancer recurrence or second primary cancers) that occurred up to 31 December 2016. Risk of death relative to the general population was approximated using standardised mortality ratios (SMRs). Treatment received was stratified according to the intensity of treatment rating, version 3 (ITR-3)., Results: During the study period, 82 noncancer disease-related deaths were recorded among 13 432 childhood cancer survivors, four times higher than expected (SMR = 4.43, 95% CI = 3.57-5.50). A clear link to treatment intensity was observed, with the relative risk of noncancer disease-related mortality being twice as high for children who underwent 'most intensive' treatment (SMR = 5.94, 95% CI = 3.69-9.55) compared to the 'least intensive' treatment group (SMR = 2.98, 95% CI = 1.42-6.24; P trend  = .01). Thirty-year cumulative mortality from noncancer disease-related deaths was estimated at 1.4% (95% CI = 1.1-1.9) after adjusting for competing causes of death such as cancer, accidents, or injuries., Conclusions: Although childhood cancer survivors are at increased relative risk of death from noncancer diseases, particularly those who undergo more intensive treatment, the cumulative mortality within 30 years of diagnosis remains small. Knowledge of late effects can guide surveillance of survivors and treatment modification, without wanting to compromise the high rates of survival., (© 2020 Wiley Periodicals LLC.)

Background: Presenting features, biology and outcome for childhood leukaemia are known to vary by ethnic origin, geographic location and socioeconomic group. This study aimed to compare presentation patterns, follow-up and clinical outcomes in Indigenous and non-Indigenous children with acute leukaemia in Australia, and to assess the impact of remoteness and area-based socioeconomic disadvantage on outcome., Methods: A retrospective review of children aged between 1 day and 18 years who were diagnosed with acute leukaemia in South Australia (SA), Northern Territory (NT) and Western Australia (WA) between 2009 and 2018 was performed. Data were collected from children treated at the Women's and Children's Hospital, Adelaide and Perth Children's Hospital., Results: Analysis of 455 children treated for acute leukaemia showed that children from remote/very remote localities had inferior overall survival (p = .004). Five-year overall survival was 91.7% (95% CI: 87.9-94.3%) for children with acute lymphoblastic leukaemia (ALL) and 69.8% (56.7-79.5%) for acute myeloid leukaemia (AML). A larger proportion of Indigenous children from SA/NT were diagnosed with AML compared to non-Indigenous children (60.0% vs. 14.4%, p = .001). Indigenous children were less likely to be enrolled on clinical trials (34.5% vs. 53.1%, p = .03) and more likely to be lost to follow-up (26.1% vs. 9.2%, p = .009)., Conclusion: Geographic remoteness of residence is associated with inferior overall survival for Australian children with leukaemia. Indigenous children with acute leukaemia suffer from disparities in outcomes. These findings provide evidence to guide national policy in supporting appropriate resource allocation to overcome the challenges faced by children within these groups., (© 2021 Wiley Periodicals LLC.)

Population-based data on survival from childhood cancers in sub-Saharan Africa are sparse. We report data on 221 children with cancer diagnosed between 2010 and 2014 in the population of Kampala, Uganda. Survival for eight of nine children with cancer assessed was below the WHO's global target of 60% (the exception was Hodgkin lymphoma: 86% at 3 years). There was significant (P < .05) decline in survival between 1 and 3 years for Wilms tumour and Kaposi sarcoma (30% and 34% at 3 years, respectively). Survival from Burkitt lymphoma, Wilms tumour and Kaposi sarcoma has not changed compared with results from the 2005-2009 study., (© 2020 Wiley Periodicals LLC.)

Background: Information on stage at diagnosis for childhood blood cancers is essential for surveillance but is not available on a population basis in most countries. Our aim was to apply the internationally endorsed Toronto Paediatric Cancer Stage Guidelines to children (<15 years) with acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), Hodgkin lymphoma (HL), or non-Hodgkin lymphoma (NHL) and to assess differences in survival by stage at diagnosis., Procedure: Stage was defined by extent of involvement of the central nervous system (CNS) for ALL and AML and using the Ann Arbor and St Jude-Murphy systems for HL and NHL, respectively. The study cohort was drawn from the population-based Australian Childhood Cancer Registry, consisting of children diagnosed with one of these four blood cancers between 2006 and 2014 with follow-up to 2015. Five-year observed survival was estimated from the Kaplan-Meier method., Results: Stage was assigned to 2201 of 2351 eligible patients (94%), ranging from 85% for AML to 95% for ALL, HL, and NHL. Survival following ALL varied from 94% (95% CI = 93%-95%) for CNS1 disease to 89% (95% CI = 79%-94%) for CNS2 (P = 0.07), whereas for AML there was essentially no difference in survival between CNS - (77%) and CNS + disease (78%; P = 0.94). Nearly all children with HL survived for five years. There was a trend (P = 0.04) toward worsening survival with higher stage for NHL., Conclusions: These results provide the first population-wide picture of the distribution and outcomes for childhood blood cancers in Australia by extent of disease at diagnosis and provide a baseline for future comparisons., (© 2019 Wiley Periodicals, Inc.)

Background: Therapy-related acute myeloid leukemia (t-AML) is defined as AML that develops after exposure to cytotoxic chemotherapy and/or radiation therapy. There is a paucity of available literature, particularly in regard to t-AML following childhood cancer. Our aim was to describe the risk of t-AML among children treated for other cancers and their subsequent survival., Procedure: We utilized data from the population-based Australian Childhood Cancer Registry to examine all childhood patients (<15 years at diagnosis) treated with chemotherapy and/or radiotherapy for cancers other than AML who received a subsequent diagnosis of AML between 1983 and 2014. Standardized incidence ratios (SIRs) were calculated to approximate the relative risk of being diagnosed with AML compared to the general population. Estimates of 5-year observed survival were obtained using the Kaplan-Meier method, with differences determined by the log-rank test., Results: Fifty-eight of 11,753 patients in the study cohort (0.5%) were diagnosed with t-AML, an almost 50-fold higher risk than expected (SIR = 45.6, 95% confidence interval [CI] = 35.3-59.0). Five-year observed survival from the date of t-AML diagnosis was 31.2% (95% CI = 19.6-43.5%). A significant survival advantage was found for patients who underwent hematopoietic stem cell transplantation (HSCT) following diagnosis of t-AML, with a 5-year survival of 52.4% (29.7-70.9%) compared to 5.7% (0.4-22.6%) for those who did not have HSCT (P < 0.001)., Conclusions: Although rare, t-AML is an important potential late effect of childhood cancer therapy. Prognosis is generally poor, with HSCT offering some survival benefit., (© 2018 Wiley Periodicals, Inc.)

Childhood acute myeloid leukaemia (AML) requires intensive therapy and is associated with survival rates that are substantially inferior to many other childhood malignancies. We undertook a retrospective analysis of Australian Paediatric Cancer Registry data from 1997 to 2008 together with a single-centre audit during the same period assessing burden on service delivery at a tertiary children's hospital (Royal Children's Hospital, Brisbane). Although survival improved from 54.3% (1997-2002) to 69.2% (2003-2008), childhood AML caused a disproportionate number of childhood cancer deaths, accounting for 5.5% of all childhood cancer diagnoses yet 7.9% of all childhood cancer mortality. Furthermore, treatment was associated with significant toxicity requiring intensive use of local health resources. Novel therapeutic strategies aimed at improving survival and reducing toxicity are urgently required., (© 2015 Wiley Periodicals, Inc.)

We report cancer incidence and mortality among Indigenous children in Australia and compare the results with corresponding data for non-Indigenous children. This information is important in understanding the overall burden of cancer in this population, and where disparities exist, to plan what action is required. Age-standardized rates, and indirectly standardized incidence and mortality ratios (SIRs and SMRs) were calculated for the years 1997-2008. There were 224 cancers identified among Indigenous children (99.5 per million per year) and 52 Indigenous children died from cancer during the study period (22.9 per million per year). The SIR for all cancers was 0.64 (95% CI = 0.56-0.73; P < 0.001) while the SMR was 0.81 (95% CI = 0.61-1.07). These results provide a baseline with which to monitor cancer among Indigenous children over time., (Copyright © 2012 Wiley Periodicals, Inc.)

Background: International studies examining the association between the incidence of childhood cancer and characteristics of the area in which the patient lives have generally reported inconsistent patterns. Area-based differentials in childhood cancer throughout Australia have not been previously published at a national level., Procedure: Population-based information from the Australian Paediatric Cancer Registry was used to identify all children aged 0- to 14-years old diagnosed with invasive cancer or intracranial and intraspinal tumors of benign or uncertain behavior between 1996 and 2006. Age-standardized incidence rates per million children per year and the corresponding incidence rate ratios were calculated, categorized by remoteness of residence and an area-based index of socioeconomic disadvantage. Results were also stratified by the most common types of childhood cancer., Results: There was a significant, decreasing gradient in the incidence of childhood cancer as remoteness of residence increased. Children living in remote or very remote areas were 21% less likely to be diagnosed with cancer compared to children in major cities, mainly due to differences in the incidence of leukemias and lymphomas. This differential was no longer significant when only non-Indigenous children were considered. No clear relationship was found between incidence and socioeconomic status (SES) in contrast to similar earlier studies., Conclusions: The findings by remoteness of residence are consistent with the lower incidence rates of cancer that are typically associated with Indigenous Australians. There is also a suggestion that the etiological factors associated with childhood leukemia and SES may have altered over time., (Copyright © 2011 Wiley Periodicals, Inc.)