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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.

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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 Science, and Embase using permutations of terms referring to childhood, cancer, survivors, prevalence, registries, and Europe. We followed PRISMA-ScR guidelines to select studies and The Joanna Briggs Institute Prevalence Critical Appraisal Tool to evaluate their quality. From 979 unique studies published between 1989 and 2022, 12 were included. Limited-duration prevalence (LDP) for all childhood cancers, assessed in three studies using counting method, varied between 450 and 1240 persons per million. Complete prevalence (CP) of survivors of any childhood cancer except skin carcinomas, reported in three studies using observed data complemented with modelled data for the unobserved period, varied between 730 and 1110 persons per million. CP of survivors of an embryonal tumour was estimated by completeness index method in six studies. In four of them CP ranged from 48 to 95 persons per million for all embryonal tumours, while CP for those occurring in central nervous system was 43 per million in one study and CP for rhabdomyosarcoma was 17 per million in another. Information on prevalence of CCS in Europe is fragmented and inconsistent. The large variations in LDP and CP estimates were linked to differences in data availability, the selection of populations, prevalence measure, statistical method, incidence period, index date, age at diagnosis and prevalence, cancer types, sex, and, for LDP, also the length of follow-up. Standardisation of methodology and reporting are needed to systematically monitor and compare CCS prevalence in Europe and provide data to help address survivors' needs.

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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.

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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.

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BACKGROUND: The Toronto Paediatric Cancer Stage Guidelines are a compendium of staging systems developed to facilitate collection of consistent and comparable data on stage at diagnosis for childhood cancers by cancer registries. MATERIAL AND METHODS: This retrospective, observational cohort study investigated changes in stage-specific incidence and survival for children diagnosed between 2000-2008 compared to 2009-2017 using the population-based Australian Childhood Cancer Registry. Information on mortality for each patient was available to 31st December 2020. Shifts in incidence by stage were evaluated using chi-square tests, and differences in stage-specific five-year observed survival for all causes of death over time were assessed using flexible parametric models. RESULTS: Stage was assigned according to the Toronto Guidelines for 96% (n = 7944) of the total study cohort (n = 8292). Changes in the distribution of incidence by stage between the two diagnosis periods were observed for retinoblastoma, with stage 0 increasing from 26% to 37% of cases (p = 0.02), and hepatoblastoma, with metastatic disease increasing from 22% to 39% of cases (p = 0.04). There were large gains in stage-specific survival over time for stage IV rhabdomyosarcoma (five-year adjusted mortality hazard ratio for 2009-2017 compared to 2000-2008 of 0.38, 95% CI 0.19-0.77; p = 0.01), stage M3 for medulloblastoma (HR = 0.41, 95% CI 0.21-0.79; p = 0.01) and metastatic neuroblastoma excluding stage MS (HR = 0.61, 95% CI 0.44-0.84; p < 0.01). CONCLUSION: These results indicate that improvements in childhood cancer survival in Australia are most likely due to refined management rather than changes in stage at diagnosis, particularly for metastatic solid tumours. Wide international uptake of the Toronto Guidelines will allow comprehensive evaluation of differences in survival between countries.

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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.

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BACKGROUND: Given the high incidence of melanoma in Australia alongside high mortality with later stage disease, we investigated the populations and locations most at risk, to optimise public health activities in areas where intervention is most needed. This study examines trends and identifies significant prognostic factors and potential disparities in incidence, mortality and survival between population groups in Victoria, Queensland and South Australia. METHODS: The analysis includes data from the population-based cancer registries of the three states over a twenty-year period (1997-2016). Age-standardized and age-specific incidence rates were calculated, and long-term trends analysed using Joinpoint Regression. Five-year relative survival estimates for the study population were calculated using the cohort method and multivariable flexible parametric survival models were applied for each jurisdiction to calculate adjusted excess mortality hazard ratios for the key characteristics. RESULTS: There were more males with melanoma than females in all the three states. Over 60% of the cases occurred in the 40-74 years age group. Most melanomas had a Breslow thickness less than or equal to 1.0 mm. For males, Victoria and Queensland had a statistically significant increasing trend whereas in South Australia there was a decreasing trend. For females, the incidence rate trend was stable in Victoria but significantly decreasing in South Australia. In Queensland there was an increasing and statistically significant trend from 2006 to 2016. Across all three states there was a reducing incidence rate in the youngest cohort, stabilizing incidence in the 40-59-year-old age group, and increasing in the oldest cohorts. Five-year relative survival decreased with increasing age and with Breslow thickness across all three jurisdictions. Males had between 43%- 46% excess mortality compared to females in all the three states. There was higher risk with increasing age and Breslow thickness, with the largest risk among the 75 + age group and those with a Breslow thickness of > 4 mm. CONCLUSION: It is the first time that data from these three registries has been analysed together in a uniform way, covering more than half of the Australian population. This study compares the epidemiology of melanoma across three states and provides a better understanding of trends and factors affecting outcome for Australians with melanoma. While there has been some improvement in aspects of incidence and mortality, this has not been evenly achieved across Australia.

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BACKGROUND: Large improvements in childhood cancer survival have been reported over recent decades. Data from cancer registries have the advantage of providing a 'whole of population' approach to gauge the success of cancer control efforts. OBJECTIVES: The aim of this study was to investigate recent survival estimates for children diagnosed with cancer Australia and to examine the extent of changes in survival over the last 35 years. For the first time, we also estimated the number of deaths among Australian children that were potentially avoided due to improvements in survival. METHODS: A retrospective, population-based cohort study design was used. Case information was extracted from the Australian Childhood Cancer Registry for 1983-2016, with follow-up to 31 December 2017. Eligible children were aged 0-14 with a basis of diagnosis other than autopsy or death certificate only. Five-year relative survival was calculated using the semi-complete cohort method for three diagnosis periods (1983-1994, 1995-2006 and 2007-2016), and changes in survival over time were assessed via flexible parametric models. Avoided deaths within 5 years for those diagnosed between 1995 and 2016 were estimated under the assumption that survival rates remained the same as for 1983-1994. RESULTS: Overall 5-year survival within the study cohort (n = 20,871) increased from 72.8% between 1983 and1994 to 86.1% between 2007 and 2016, equating to an adjusted excess mortality hazard ratio of 1.82 (95% confidence interval 1.67, 1.97). Most cancers showed improvements in survival; other gliomas, hepatoblastoma and osteosarcoma were exceptions. Among children diagnosed between 1995 and 2016, 38.7% of expected deaths within 5 years of diagnosis (n = 1537 of 3970) were avoided due to temporal improvements in survival. CONCLUSIONS: Survival for childhood cancer has continued to improve over recent years, thanks mainly to ongoing progress in treatment development combined with improved supportive care. Providing innovative measures of survival, such as avoided deaths, may assist with understanding outcome data produced by cancer registries.

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Purpose: Increased risk of second primary cancers is an unwanted consequence of cancer survivorship. While the epidemiology of second cancers is well-documented for children and older people, less is known about second cancers among adolescent and young adult (AYA) cancer survivors. Methods: Unit record data were obtained from the Queensland Cancer Register. The study cohort comprised Queensland residents aged 15 to 39 years who were diagnosed with a first primary invasive cancer between 1982 and 2013. Follow-up on second cancers was available for a minimum of 5 years to the end of 2018. Standardized incidence ratios (SIRs) were used to approximate the risk of a second primary cancer relative to the general population. Results: In total, 3086 second primary cancers were observed among 34,431 eligible AYA patients (9%), equating to an overall SIR of 1.59 (95% confidence interval [CI] 1.53-1.64). Melanoma (n = 853, 28%) and female breast cancer (n = 594, 19%) were the most common types of second primary cancer in the study cohort. Relative risk of all second primary cancers combined among AYA patients was inversely associated with age and was highest within the period immediately after first diagnosis irrespective of age group; for example, patients aged 15-24 at first diagnosis recorded more than four times as many second primary cancers than expected within 2 years of their first cancer (SIR = 4.40, 95% CI 2.83-6.82). Conclusions: Detailed data on second primary cancers among AYA cancer survivors are important in promoting increased awareness and to inform the development of targeted prevention and surveillance strategies.

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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.

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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.

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PURPOSE: To investigate incidence and survival of childhood tumours of the central nervous system (CNS) by histological subtype, tumour behaviour and tumour grade. METHODS: National, population-based data on all children under 15 years old diagnosed with a CNS tumour between 1983 and 2016 were sourced from the Australian Childhood Cancer Registry. Incidence rate trends were calculated using Joinpoint regression. Relative survival was calculated using the cohort method, with changes in survival over time by cancer type and tumour grade assessed by multivariable flexible parametric survival modelling. RESULTS: The study cohort included 4914 patients, with astrocytoma (n = 2181, 44%) and embryonal tumours (n = 931, 19%) the most common diagnostic subgroups. Almost half (n = 2181, 44%) of all tumours were classified as high grade (III or IV). Incidence rates increased by 29% between 1983 and 2016, with high grade tumours rising by an annual average of + 1.1% (95% CI = + 0.7%, + 1.5%, p < 0.001). 5-year survival for all patients combined was 72% (95% CI = 71-74%), ranging from 50% (46-54%) for those with other gliomas to 81% (79-83%) for astrocytoma (p < 0.001). Survival improved over time for grade II and III ependymomas but not for patients with astrocytoma irrespective of grade. CONCLUSION: Improvements in diagnostic technology leading to more precise tumour classification are likely to explain some of the differences in incidence rate trends by histological type and grade. While improvements in survival over time were noted for some tumours, outcomes remained poor among patients with high-grade astrocytoma.

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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.

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The lack of accurate population-based information on childhood cancer stage and survival in low-income countries is a barrier to improving childhood cancer outcomes. In our study, data from three population-based registries in sub-Saharan Africa (Abidjan, Harare and Kampala) were examined for children aged under 15. We assessed the feasibility of assigning stage at diagnosis according to Tier 1 of the Toronto Childhood Cancer Stage Guidelines for patients with non-Hodgkin lymphoma [including Burkitt lymphoma (BL)], retinoblastoma and Wilms' tumour. Patients were actively followed-up, allowing calculation of 3-year relative survival by cancer type and registry. Stage-specific observed survival was estimated. The cohort comprised 381 children, of whom half (n = 192, 50%) died from any cause within 3 years of diagnosis. Three-year relative survival varied by malignancy and location and ranged from 17% [95% confidence interval (CI) = 6%-33%] for BL in Harare to 57% (95% CI = 31%-76%) for retinoblastoma in Kampala. Stage was assigned for 83% of patients (n = 317 of 381), with over half having metastatic or advanced disease at diagnosis (n = 166, 52%). Stage was a strong predictor of survival for each malignancy; for example, 3-year observed survival was 88% (95% CI = 68%-96%) and 13% (4%-29%) for localised and advanced BL, respectively (P < .001). These are the first data on stage distribution and stage-specific survival for childhood cancers in Africa. They demonstrate the feasibility of the Toronto Stage Guidelines in a low-resource setting and highlight the value of population-based cancer registries in aiding our understanding of the poor outcomes experienced by this population.

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BACKGROUND: Pediatric hepatic cancer is a rare malignancy, comprising only approximately 2% of all cancers diagnosed in children aged <15 years. The authors sought to describe trends in pediatric hepatic cancer incidence and survival in Ontario, Canada; the United States; and Australia. METHODS: Children aged <15 years who were diagnosed with hepatic cancer from 1985 through 2013 were ascertained through population-based registries and followed from the time of diagnosis until December 31, 2015. Age-standardized incidence and 5-year relative survival were calculated for each jurisdiction. Multivariable flexible parametric survival models were used to explore predictors of hepatic cancer mortality. RESULTS: A total of 794 children were identified in Ontario (148 children), the United States (400 children), and Australia (246 children). The average annual incidence increased by 2.2% (95% CI, 0.5%-4.0%) in Australia, 2.1% (95% CI, 0.9%-3.3%) in the United States, and 1.3% (95% CI, -0.4% to 3.0%) in Ontario. The 5-year relative survival rate improved from 60% to 82% (P = .08) in Ontario and 62% to 78% (P = .02) in the United States between the diagnostic periods 1985 through 1994 and 2005 through 2013, whereas in Australia the rate remained constant (between 74% and 77%) during the study period. On multivariable analysis, there was no significant difference noted with regard to the hazard of death between jurisdictions (P = .06). Older age, the presence of metastatic disease, and being diagnosed with hepatocellular carcinoma were found to be associated with mortality. CONCLUSIONS: The incidence of hepatic cancer in children appears to have increased over the last 30 years in Australia and North America. Survival differences between Australia; Ontario, Canada; and the United States observed in the 1980s and 1990s were no longer apparent and only marginal geographical differences in the hazard of mortality were observed.

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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; Ptrend  = .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.

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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.

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BACKGROUND: The literature surrounding survival of patients with multiple primary melanomas (MPM) yields variable and opposing findings, constrained by statistical challenges. OBJECTIVES: To critically examine the available literature regarding survival of patients with MPM compared with a single primary melanoma and detail statistical methods used. METHODS: Electronic searches were performed of PubMed, Embase, Web of Science, and Scopus, with cross-checking of references, for the period January 1956 to June 2019. Studies published in English examining survival in patients with multiple melanomas were included. Case studies and small case series were excluded. RESULTS: There were 14 studies eligible for inclusion. Conclusions on survival varied markedly depending on the statistical method used. Four studies that accounted for survival bias by partitioning the survival time were included in the quantitative review, with 3 of these reporting a survival disadvantage for MPM, whereas the fourth showed no difference in survival. The pooled hazard ratio was 1.39 (95% confidence interval, 1.07-1.81) but with significant heterogeneity (I2 = 96.8%, Phet < .001). LIMITATIONS: Studies showed significant heterogeneity in methodology. CONCLUSION: When data were analyzed with robust statistical methods, patients with MPM had a survival disadvantage compared with patients with a single primary melanoma.

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BACKGROUND: Childhood liver cancers are relatively rare, hence inferences on incidence trends over time are limited by lack of precision in most studies. OBJECTIVE: To conduct a systematic review and meta-analysis of published contemporary trends on childhood liver cancer incidence rates worldwide. DATA SOURCES: PubMed, EMBASE, CINAHL, Web of Science. STUDY SELECTION AND DATA EXTRACTION: English-language peer-reviewed articles published from 1 January 2008 to 1 December 2019 that presented quantitative estimates of incidence trends for childhood liver cancer and diagnostic subgroups. Review was conducted per PRISMA guidelines. Two authors independently extracted data and critically assessed studies. SYNTHESIS: Random effects meta-analysis models were used to estimate pooled incidence trends by diagnostic subgroups. Heterogeneity was measured using the Q and I2 statistics and publication bias evaluated using Egger's test. RESULTS: Eighteen studies were included, all based on population-based cancer registries. Trends were reported on average for 18 years. Overall pooled estimates of the annual percentage change (APC) were 1.4 (95% confidence interval [CI] 0.5, 2.3) for childhood liver cancers, 2.8 (95% CI 1.8, 3.8) for hepatoblastoma and -3.0 (95% CI -11.0, 4.9) for hepatocellular carcinoma. Sub-group analysis by region indicated increasing trends for childhood liver cancers in North America/Europe/Australia (APC 1.7, 95% CI 0.7, 2.8) whereas corresponding trends were stable in Asia (APC 1.4, 95%CI -0.3, 2.7). Publication bias was not detected for any of these analyses. The I2 statistic indicated that the heterogeneity among included studies was low for combined liver cancers, moderate for hepatoblastoma and high for hepatocellular carcinoma. CONCLUSIONS: Incidence is increasing for childhood liver cancers and the most commonly diagnosed subgroup hepatoblastoma. Lack of knowledge of the etiology of childhood liver cancers limited the ability to understand the reasons for observed incidence trends. This review highlighted the need for ongoing monitoring of incidence trends and etiological studies.

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