Childhood Cancer In Australia Research Articles

A review of Leila Rose Foundation support for families affected by rare childhood cancer in Australia over the past decade.

BackgroundThe Leila Rose Foundation (“the Foundation”) was established in April 2011, to address financial toxicity as well as the gaps in knowledge and support for families affected by a rare childhood cancer diagnosis in Australia.AimThe aim of this brief report is to analyze the diagnostic trends surrounding the rare cancer diagnoses for patients referred to the Foundation over the past decade and to present case studies evaluating the role of the Foundation's Family Support Coordinator in providing tailored, individualized support for families.MethodsEligibility for family support is restricted to children ≤ 14 years of age at diagnosis with a cancer that has an incidence less than 5% of all childhood cancers in Australia as reflected by national registry data. The analysis of diagnostic trends in this report, was based upon a systematic review of enrolment records. The role of the Family Support Coordinator is presented in four different case studies.ResultsAs at 1 November 2020, the Foundation has supported 197 families affected by rare childhood cancer. Financial support of $825,000 has been provided directly to these families. Enrollment records demonstrate that 35 patients representing 18% of all enrollments have had a unique diagnosis that has not been recorded for any other enrolled patient highlighting that these diagnoses are very rare. The most frequent diagnoses have included Medulloblastoma, Ewing's Sarcoma and Wilm's Tumor (20, 19, 19 patients respectively). The Family Support Coordinator role has provided individualized support for families which has been greatly appreciated based upon ad hoc family feedback.ConclusionsChallenges remain in terms of improving outcomes for families affected by rare childhood cancer. The Foundation is committed to leaving no stone unturned and delivering its unique support services to families in order to reduce the burden caused by a rare childhood cancer diagnosis both now and in the future.

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

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. 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 5years, 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). During the study period, 82 noncancer disease-related deaths were recorded among 13432 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. 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 30years 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.

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

ObjectivesTo describe changes in childhood cancer incidence in Australia, 1983–2015, and to estimate projected incidence to 2035.Design, settingPopulation‐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 measuresIncidence 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.ResultsThe 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).ConclusionsThe 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.

Abstract B42: Hope for a future: Conditional survival estimates for childhood cancer in Australia, 2002-2011

Abstract Purpose: Conditional survival rates provide a more realistic assessment of long-term prognosis than survival rates calculated from the date of diagnosis because they take into account the time that a patient has already remained alive following diagnosis. However, estimates of conditional survival are scarce for childhood cancer. Here we provide results from a national population-based childhood cancer registry by diagnostic group, age at diagnosis and stage of cancer. Methods: De-identified population-based data were obtained from the Australian Paediatric Cancer Registry for children aged 0-14 years diagnosed with cancer between 1983 and 2010. Mortality status was followed up to the end of 2011. The hybrid period method was used to calculate relative survival estimates for those who were at risk during the period 2002 to 2011. Conditional survival was then obtained from the ratio of the relative survival estimates at different time points. Results: A total of 13,537 children were eligible for inclusion in the study. Five-year survival for all childhood cancers combined improved from 82% at diagnosis (95% confidence interval = 81%-83%) to 89% (88%-90%), 95% (94%-96%) and 97% (97%-98%) conditional on surviving 1, 3 and 5 years after diagnosis, respectively. Conditional survival reached 95% within five years of diagnosis for nearly all types of cancer, regardless of age or stage at diagnosis. Conclusions: Most children diagnosed with cancer who are alive five years after diagnosis can anticipate a similar chance of survival to children in the general population. For childhood cancer patients and their families, this information provides a more accurate and optimistic picture of long-term prognosis than has been available until now. Citation Format: Danny Youlden, Peter Baade, Andrew Hallahan, Patricia Valery, Adele Green, Joanne Aitken. Hope for a future: Conditional survival estimates for childhood cancer in Australia, 2002-2011. [abstract]. In: Proceedings of the AACR Special Conference on Advances in Pediatric Cancer Research: From Mechanisms and Models to Treatment and Survivorship; 2015 Nov 9-12; Fort Lauderdale, FL. Philadelphia (PA): AACR; Cancer Res 2016;76(5 Suppl):Abstract nr B42.

Conditional survival estimates for childhood cancer in Australia, 2002–2011: A population-based study

BackgroundConditional survival estimates take into account the time that a patient has remained alive following diagnosis to provide a realistic perspective on the probability of longer term survival. Such estimates are scarce for childhood cancer, particularly by age at diagnosis or stage of cancer. MethodsDe-identified population-based data were obtained from the Australian Paediatric Cancer Registry for children aged 0–14 years diagnosed with cancer between 1983 and 2010. Mortality status was followed up to the end of 2011. The hybrid period method was used to calculate relative survival estimates for those who were at risk during the period 2002–2011. Conditional survival stratified by diagnostic group or subgroup, age and stage at diagnosis was then obtained from the ratio of the relative survival estimates at different time points. ResultsA total of 13,537 children were eligible for inclusion. Five-year survival for all childhood cancers combined improved from 82% at diagnosis (95% confidence interval=81–83%) to 89% (88–90%) conditional on surviving one year, and 97% (97–98%) conditional on surviving five years after diagnosis. Conditional survival reached 95% within five years of diagnosis for nearly all types of cancer, regardless of a child's age or stage at diagnosis. ConclusionMost children diagnosed with cancer who are alive five years after diagnosis can anticipate similar survival to children in the general population. This information may help alleviate some of the distress associated with childhood cancer, particularly for those with an initially poor prognosis.

Differentials in Survival for Childhood Cancer in Australia by Remoteness of Residence and Area Disadvantage

It is not known whether improvements in cancer survival over recent decades have benefited children from different geographic locations equally. This is the first study to produce national survival estimates for childhood cancer in Australia by remoteness of residence and area-based socioeconomic status. The study utilized population-based data from the Australian Paediatric Cancer Registry for children diagnosed with cancer from 1996 onward who were at risk of mortality between January 2001 and December 2006 (n = 6,289). Remoteness was specified according to the Australian Standard Geographical Classification Remoteness Areas, whereas an index of area disadvantage was obtained from census information. Five-year relative survival estimates were produced by the period method for all cancers and the most common diagnostic groups, with corresponding age-sex adjusted mortality hazard ratios calculated using Poisson regression. Overall, children with cancer from remote/very remote areas had a significantly lower survival rate than their counterparts in major cities (HR = 1.55, 95% CI = 1.08-2.23). Survival was also lower for children with leukemia living in inner regional (HR = 1.52, 95% CI = 1.11-2.08) or outer regional areas (HR = 1.53, 95% CI = 1.03-2.28). There was weak evidence (P(grad) = 0.051) of a trend toward poorer survival by greater area disadvantage for all childhood cancers. Some variation in prognosis by place of residence was present for children with cancer in Australia, particularly among leukemia patients. Treatment, clinical or area-related factors that contribute to these survival differentials need to be identified.

Population-based survival estimates for childhood cancer in Australia during the period 1997–2006

Background:This study provides the latest available relative survival data for Australian childhood cancer patients.Methods:Data from the population-based Australian Paediatric Cancer Registry were used to describe relative survival outcomes using the period method for 11 903 children diagnosed with cancer between 1983 and 2006 and prevalent at any time between 1997 and 2006.Results:The overall relative survival was 90.4% after 1 year, 79.5% after 5 years and 74.7% after 20 years. Where information onstage at diagnosis was available (lymphomas, neuroblastoma, renal tumours and rhabdomyosarcomas), survival was significantly poorer for more-advanced stage. Survival was lower among infants compared with other children for those diagnosed with leukaemia, tumours of the central nervous system and renal tumours but higher for neuroblastoma. Recent improvements in overall childhood cancer survival over time are mainly because of improvements among leukaemia patients.Conclusion:The high and improving survival prognosis for children diagnosed with cancer in Australia is consistent with various international estimates. However, a 5-year survival estimate of 79% still means that many children who are diagnosed with cancer will die within 5 years, whereas others have long-term health morbidities and complications associated with their treatments. It is hoped that continued developments in treatment protocols will result in further improvements in survival.

Late mortality and second cancers in an Australian cohort of childhood cancer survivors

The aim of this study was to characterise rates of late mortality and second cancers in an Australian cohort of childhood cancer survivors and compare these to rates observed in the New South Wales population. Records for 896 childhood cancer survivors treated at the Sydney Children's Hospital between 1972 and 1999 were linked to the National Death Index and NSW Central Cancer Registry to identify deaths and notifications of second cancers. Survivors were defined as those alive for at least 5 years after diagnosis and were followed until death or 31 December 2004, whichever occurred first. Standardised mortality ratios (SMRs) and standardised incidence ratios (SIRs) were used as measures of relative risk. A Cox proportional hazard model was used to quantify the influence of demographic and disease-related characteristics on the risk of death and second cancers. The SMR and SIR were 7.46 and 4.98 times higher, respectively, among cancer survivors relative to the NSW population. Relative mortality was highest in survivors of soft-tissue sarcoma (SMR, 18.95 [95% CI, 6.88-40.81]) and central nervous system (CNS) malignancies (SMR, 16.78 [95% CI, 7.62-31.64]). The leading causes of death included recurrence of the primary childhood cancer (55%) and second cancers (12%), as well as treatment-related complications (17%) The most frequently observed second cancers were bone and thyroid cancers, melanoma, and CNS malignancies, and second cancers were most common among survivors of leukaemia, soft-tissue sarcoma and Hodgkin's lymphoma. Compared with the general population, survivors of childhood cancer in Australia are at increased risk of late mortality and second cancers. These findings highlight a continuing need to assess health issues faced by childhood cancer survivors and develop strategies to minimise the adverse outcomes associated with treatment for childhood cancer.

Trends in incidence of childhood cancer in Australia, 1983–2006

Background:There are few population-based childhood cancer registries in the world containing stage and treatment data.Methods:Data from the population-based Australian Paediatric Cancer Registry were used to calculate incidence rates during the most recent 10-year period (1997–2006) and trends in incidence between 1983 and 2006 for the 12 major diagnostic groups of the International Classification of Childhood Cancer.Results:In the period 1997–2006, there were 6184 childhood cancer (at 0–14 years) cases in Australia (157 cases per million children). The commonest cancers were leukaemia (34%), that of the central nervous system (23%) and lymphomas (10%), with incidence the highest at 0–4 years (223 cases per million). Trend analyses showed that incidence among boys for all cancers combined increased by 1.6% per year from 1983 to 1994 but have remained stable since. Incidence rates for girls consistently increased by 0.9% per year. Since 1983, there have been significant increases among boys and girls for leukaemia, and hepatic and germ-cell tumours, whereas for boys, incidence of neuroblastomas and malignant epithelial tumours has recently decreased. For all cancers and for both sexes combined, there was a consistent increase (+0.7% per year, 1983–2006) at age 0–4 years, a slight non-significant increase at 5–9 years, and at 10–14 years, an initial increase (2.7% per year, 1983–1996) followed by a slight non-significant decrease.Conclusion:Although there is some evidence of a recent plateau in cancer incidence rates in Australia for boys and older children, interpretation is difficult without a better understanding of what underlies the changes reported.

Tracing survivors of childhood cancer in Australia

A major challenge when establishing a retrospective cohort of childhood cancer survivors is maximizing the enrolment of individuals treated as children. In Australia, several public databases can facilitate tracing of childhood cancer survivors; these include the Births, Deaths and Marriage Registers (BDMR), the telephone white pages (EWP), and the Australian Electoral Roll (AER). The aim of this study was to evaluate the characteristics of survivors traced for a cohort study examining the late effects of childhood cancer. Eligible participants included 1,198 individuals treated for cancer during childhood at Sydney Children's Hospital. Of these, 1,156 individuals were confirmed alive and subsequently traced by cross matching against the AER, BDMR, WP, and clinical records. Characteristics influencing the likelihood of tracing were evaluated using logistic regression analyses. Current addresses were obtained for 810 (70.1%) survivors. Overall, those diagnosed within the last decade were more easily traced than those diagnosed in an earlier decade (Test for trend, P < 0.01). In addition, older age at diagnosis and diagnosis of a sympathetic nervous system or renal malignancy were associated with a decreased likelihood of tracing in survivors, aged >/=18 years (P < 0.05). In survivors aged <18 years, age at diagnosis, sex, and cancer diagnosis were not associated with the likelihood of tracing (P > 0.05). Our findings show that cancer type and year of diagnosis are independent factors influencing the ability to trace survivors of childhood cancer. Linkage to public databases provides a unique opportunity to recruit a representative hospital-based cohort of childhood cancer survivors in Australia.

Electronic Telephone Directory Listings: Preferred Sampling Frame for a Population-Based Study of Childhood Cancer in Australia

PURPOSE: We report our telephone-based system for selecting community control series appropriate for a complete Australia-wide series of Ewing's sarcoma cases. METHODS: We used electronic directory random sampling to select age-matched controls. The sampling has all listed telephone numbers on an up-dated CD-Rom. RESULTS: 95% of 2245 telephone numbers selected were successfully contacted. The mean number of attempts needed was 1.94, 58% answering at the first attempt. On average, we needed 4.5 contacts per control selected. Calls were more likely to be successful (reach a respondent) when made in the evening (except Saturdays). The overall response rate among contacted telephone numbers was 92.8%. Participation rates among female and male respondents were practically the same. The exclusion of unlisted numbers (13.5% of connected households) and unconnected households (3.7%) led to potential selection bias. However, restricting the case series to listed cases only, plus having external information on the direction of potential bias allow meaningful interpretation of our data. CONCLUSION: Sampling from an electronic directory is convenient, economical and simple, and gives a very good yield of eligible subjects compared to other methods.

Childhood cancer incidence in Australia, 1982-1991.

The data of the Australian Paediatric Cancer Registry on childhood cancer incidence in Australia for the 10-year period 1982-1991 are presented. The crude average annual incidence of cancer in children under the age of 15 years was 13.8 per 100,000. The incidence of childhood cancer in Australia is rising. Significant increases were seen in acute non-lymphoblastic leukaemia, astrocytoma and melanoma. The age-standardised incidence of 14.4 per 100,000 is about 34% higher than in the UK. Most types of cancer had a higher incidence in Australia than in the UK, and the difference was significant for acute lymphoblastic leukaemia, astrocytoma and melanoma. Of particular interest is malignant melanoma, whose incidence in Australia is more than 5 times that in the UK, as a result of excessive UV exposure. Australia has a higher incidence of Ewing's tumour than osteosarcoma, nearly twice that of the UK. International comparative studies may help to elucidate the aetiology of these tumours.

Childhood cancer incidence in Australia, 1982–1991

The data of the Australian Paediatric Cancer-Registry on childhood cancer incidence in Australia for the 10-year period 1982–1991 are presented. The crude average annual incidence of cancer in children under the age of 15 years was 13.8 per 100,000. The incidence of childhood cancer in Australia is rising. Significant increases were seen in acute non-lymphoblastic leukaemia, astrocytoma and melanoma. The age-standardised incidence of 14.4 per 100,000 is about 34% higher than in the UK. Most types of cancer had a higher incidence in Australia than in the UK, and the difference was significant for acute lymphoblastic leukaemia, astrocytoma and melanoma. Of particular interest is malignant melanoma, whose incidence in Australia is more than 5 times that in the UK, as a result of excessive UV exposure. Australia has a higher incidence of Ewing's tumour than osteosarcoma, nearly twice that of the UK. International comparative studies may help to elucidate the aetiology of these tumours. © 1996 Wiley-Liss, Inc.