Incidence and survival for childhood cancer by endorsed non-stage prognostic indicators in Australia.

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.

Long-term childhood cancer survival in Australia using period estimation.

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.

The growing prevalence of childhood cancer survivors in Australia.

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.

Childhood cancer survival and avoided deaths in Australia, 1983-2016.

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.

Temporal changes in childhood cancer incidence and survival by stage at diagnosis in Australia, 2000-2017.

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.

Changes in cancer incidence and survival among Aboriginal and Torres Strait Islander children in Australia, 1997-2016.

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.

Stage at diagnosis and survival by stage for the leading childhood cancers in three populations of sub-Saharan Africa.

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.

Pediatric hepatic cancer incidence and survival: 30-year trends in Ontario, Canada; the United States; and Australia.

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.

Incidence and survival for childhood central nervous system tumours in Australia, 1983-2016.

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.

Late mortality from other diseases following childhood cancer in Australia and the impact of intensity of treatment.

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.

Survival from childhood cancer in Kampala, Uganda.

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.

Are outcomes for childhood leukaemia in Australia influenced by geographical remoteness and Indigenous race?

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.

Global trends in incidence rates of childhood liver cancers: A systematic review and meta-analysis.

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.

Survival in patients with multiple primary melanomas: Systematic review and meta-analysis.

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.

Second primary cancers in people who had cancer as children: an Australian Childhood Cancer Registry population-based study.

OBJECTIVE: To investigate the incidence of second primary cancers in people diagnosed with cancer during childhood. DESIGN, SETTING: Retrospective, population-based study; analysis of Australian Childhood Cancer Registry data. PARTICIPANTS: People alive at least two months after being diagnosed before the age of 15 years with a primary cancer, 1983-2013, followed until 31 December 2015 (2-33 years' follow-up). MAIN OUTCOME MEASURES: Risks of second primary cancer compared with the general population, expressed as standardised incidence ratios (SIRs). RESULTS: Among 18 230 people diagnosed with cancer during childhood, 388 (2%) were later diagnosed with second primary cancers; the estimated 30-year cumulative incidence of second cancers was 4.4% (95% CI, 3.8-5.0%). The risk of a new primary cancer was five times as high as for the general population (SIR, 5.13; 95% CI, 4.65-5.67). Relative risk of a second primary cancer was greatest for people who had childhood rhabdomyosarcoma (SIR, 19.9; 95% CI, 14.4-27.6). Relative risk was particularly high for children who had undergone both chemotherapy and radiotherapy (SIR, 9.80; 95% CI, 8.35-11.5). Relative risk peaked during the 5 years following the first diagnosis (2 to less than 5 years: SIR, 10.3; 95% CI, 8.20-13.0), but was still significant at 20-33 years (SIR, 2.58; 95% CI, 2.02-3.30). The most frequent second primary cancers were thyroid carcinomas (65 of 388, 17%) and acute myeloid leukaemias (57, 15%). CONCLUSIONS: Survivors of childhood cancer remain at increased risk of a second primary cancer well into adulthood. As the late effects of cancer treatment probably contribute to this risk, treatments need to be refined and their toxicity reduced, without reducing their benefit for survival.

The incidence of childhood cancer in Australia, 1983-2015, and projections to 2035.

OBJECTIVES: To describe changes in childhood cancer incidence in Australia, 1983-2015, and to estimate projected incidence to 2035. DESIGN, SETTING: Population-based study; analysis of Australian Childhood Cancer Registry data for the 20 547 children under 15 years of age diagnosed with cancer in Australia between 1983 and 2015. MAIN OUTCOME MEASURES: Incidence rate changes during 1983-2015 were assessed by joinpoint regression, with rates age-standardised to the 2001 Australian standard population. Incidence projections to 2035 were estimated by age-period-cohort modelling. RESULTS: The overall age-standardised incidence rate of childhood cancer increased by 34% between 1983 and 2015, increasing by 1.2% (95% CI, +0.5% to +1.9%) per annum between 2005 and 2015. During 2011-2015, the mean annual number of children diagnosed with cancer in Australia was 770, an incidence rate of 174 cases (95% CI, 169-180 cases) per million children per year. The incidence of hepatoblastoma (annual percentage change [APC], +2.3%; 95% CI, +0.8% to +3.8%), Burkitt lymphoma (APC, +1.6%; 95% CI, +0.4% to +2.8%), osteosarcoma (APC, +1.1%; 95%, +0.0% to +2.3%), intracranial and intraspinal embryonal tumours (APC, +0.9%; 95% CI, +0.4% to +1.5%), and lymphoid leukaemia (APC, +0.5%; 95% CI, +0.2% to +0.8%) increased significantly across the period 1983-2015. The incidence rate of childhood melanoma fell sharply between 1996 and 2015 (APC, -7.7%; 95% CI, -10% to -4.8%). The overall annual cancer incidence rate is conservatively projected to rise to about 186 cases (95% CI, 175-197 cases) per million children by 2035 (1060 cases per year). CONCLUSIONS: The incidence rates of several childhood cancer types steadily increased during 1983-2015. Although the reasons for these rises are largely unknown, our findings provide a foundation for health service planning for meeting the needs of children who will be diagnosed with cancer until 2035.

Incidence and outcomes of neuroblastoma in Australian children: A population-based study (1983-2015).

AIM: Neuroblastoma predominantly affects younger children and exhibits heterogeneous behaviour. This study describes incidence and outcomes for neuroblastoma using national population-based data from the Australian Childhood Cancer Registry. METHODS: Deidentified data for all children (0-14 years) diagnosed with neuroblastoma and ganglioneuroblastoma from 1983 to 2015 were extracted. Cause-specific (CSS) and event-free survival were estimated using the cohort method. Adjusted hazard ratios were calculated using a multivariable flexible parametric survival model. Other outcomes investigated included recurrence and second primary malignancies (SPMs). RESULTS: The study cohort comprised 1269 patients. Age-standardised incidence rates remained steady across the study period at approximately 9.5 per million children per year. The proportion of patients with metastatic disease at diagnosis decreased from 63% in 1983-1995 to 42% by 2006-2015 (P < 0.001). CSS and event-free survival both improved significantly over time and reached 75% (95% confidence interval (CI) = 71-79%) and 71% (95% CI = 66-75%) at 5 years post-diagnosis, respectively, for children diagnosed between 2004 and 2013. Of patients achieving full remission, 28% relapsed with subsequent 5-year CSS of only 20%. Although SPMs were rare, neuroblastoma survivors carried a fivefold increased risk compared to cancer rates in the general population (standardised incidence ratio = 5.18, 95% CI = 3.01-8.91), with 7 of the 13 patients (54%) who were diagnosed with an SPM dying within 5 years. CONCLUSIONS: CSS for childhood neuroblastoma has improved substantially over time in Australia, but still remains lower than for most other types of childhood cancer. SPMs are uncommon and carry a better prognosis than relapse of the primary tumour.

Renal tumours in Australian children: 30 years of incidence, outcome and second primary malignancy data from the Australian Childhood Cancer Registry.

AIM: This paper describes the incidence and outcomes of childhood renal malignancies in Australia using national population-based data from the Australian Childhood Cancer Registry. METHODS: De-identified data for children (0-14 years) diagnosed with renal malignancies from 1983 to 2015 inclusive were extracted. Cause-specific (CSS) and event-free survival up to 20 years from diagnosis were estimated using the cohort method. Adjusted excess mortality hazard ratios were calculated using a multivariable flexible parametric survival model. Details relating to second primary malignancies (SPMs) were also examined. RESULTS: There were 1046 children diagnosed with renal malignancies in Australia between 1983 and 2015 (91% nephroblastoma), generating an annual age-standardised incidence rate of 8 per million children, which remained constant over the study period. CSS was 89% (95% confidence interval = 87-91%) and 88% (86-90%) at 5 and 20 years, respectively, and 5-year event-free survival was 82% (80-84%). Five-year CSS did not change over the study period and was highest for nephroblastoma (91%). Of the 94% of patients achieving remission, 15% relapsed and subsequent 5-year CSS was 49% (40%-58%). Eleven children were diagnosed with SPM (standardised incidence ratio = 2.9, 95% confidence interval = 1.6-5.3, P < 0.001), and five of them (45%) died within 5 years of the second diagnosis. CONCLUSIONS: Children treated for renal malignancies in Australia have excellent long-term survival, which is unchanged since 1983. SPMs are uncommon following treatment for childhood renal cancer but carry a poor prognosis. Relapse carries a similarly poor prognosis to SPM but is more common. These data are comparable to registry outcomes in similarly developed nations.

The impact of changing the prevalence of overweight/obesity and physical inactivity in Australia: An estimate of the proportion of potentially avoidable cancers 2013-2037.

Globally, 39% of the world's adult population is overweight or obese and 23% is insufficiently active. These percentages are even larger in high-income countries with 58% overweight/obese and 33% insufficiently active. Fourteen cancer types have been declared by the World Cancer Research Fund to be causally associated with being overweight or obese: oesophageal adenocarcinoma, stomach cardia, colon, rectum, liver, gallbladder, pancreas, breast, endometrium, ovary, advanced/fatal prostate, kidney, thyroid and multiple myeloma. Colon, postmenopausal breast and endometrial cancers have also been judged causally associated with physical inactivity. We aimed to quantify the proportion of cancer cases that would be potentially avoidable in Australia if the prevalence of overweight/obesity and physical inactivity in the population could be reduced. We used the simulation modelling software PREVENT 3.01 to calculate the proportion of avoidable cancers over a 25-year period under different theoretical intervention scenarios that change the prevalence of overweight/obesity and physical inactivity in the population. Between 2013 and 2037, 10-13% of overweight/obesity-related cancers in men and 7-11% in women could be avoided if overweight and obesity were eliminated in the Australian population. If everyone in the population met the Australian physical activity guidelines for cancer prevention (i.e. engaged in at least 300 min of moderate-intensity physical activity per week), an estimated 2-3% of physical inactivity-related cancers could be prevented in men (colon cancer) and 1-2% in women (colon, breast and endometrial cancers). This would translate to the prevention of up to 190,500 overweight/obesity-related cancers and 19,200 inactivity-related cancers over 25 years.

The impact of reducing alcohol consumption in Australia: An estimate of the proportion of potentially avoidable cancers 2013-2037.

The International Agency for Research on Cancer first concluded that alcohol causes cancer in humans in 1988. The World Cancer Research Fund has declared that alcohol causes cancer of the oral cavity, pharynx, larynx, oesophagus (squamous cell carcinoma), female breast, colon, rectum, stomach and liver. It recommended that alcohol be avoided altogether to prevent cancer. We aimed to quantify the impact of reducing alcohol consumption on future cancer incidence in Australia. We used PREVENT 3.01 simulation modelling software to estimate the proportion of cancers that could potentially be prevented over a 25-year period under two hypothetical intervention scenarios and two latency periods (20 and 30 years). Under a scenario where alcohol consumption abruptly ceases, we estimated up to 4% of alcohol-related cancers could be avoided over a 25-year period (~49,500 cancers, depending on assumed latency). If the maximum consumption of all Australian adults was ≤20 g/day (~two Australian standard drinks), up to 2% of alcohol-related cancers could be avoided (~29,600 cancers). The maximum proportions were higher for men (6% for no alcohol consumption; 5% for ≤20 g/day) than women (3%; 1%). The proportion avoidable was highest for oesophageal squamous cell carcinoma (17% no alcohol consumption; 9% ≤20 g/day), followed by cancers of the oral cavity (12%; 5%) and pharynx (11%; 5%). The cancer sites with the highest numbers of potentially avoidable cases were colon in men (11,500; 9,900) and breast in women (14,400; 4,100). Successful interventions to reduce alcohol intake could lead to significant reductions in cancer incidence.