Five-year relative survival by stage at diagnosis for childhood cancers

Published

07 Oct, 2021

Introduction:

This NCCI measure presents national data on 5-year relative survival by stage at diagnosis for 16 major childhood cancers, for the 10-year period 2006-2015. The data build upon Cancer Australia’s previous childhood cancer data releases for the periods 2006–2010 and 2006−2014, and are presented using the disease-specific staging systems for each of the childhood cancer types listed in the ‘About the data’ tab. Together, these 16 cancer types represent approximately three-quarters of all childhood cancers diagnosed in Australia and other high-income countries.¹

Five-year relative survival was determined by a follow-up on mortality status to 31 Dec 2016. The Five-year relative survival by stage at diagnosis for childhood cancers measure is one of four reported on the National Cancer Control Indicators (NCCI) website that are related to childhood cancers. The other measures are:

Distribution of childhood cancer stage
Distribution of childhood cancer stage by remoteness and socioeconomic status
Five-year survival by remoteness and socioeconomic status.

Childhood cancer stage at diagnosis has been determined using Business Rules for deriving stage based on the Toronto Paediatric Cancer Stage Guidelines.^{2, 3} More information about the Business Rules used for collection, data sources, methods for recording, and guidance for interpreting the data can be found in the ‘About the Data’ tab.

For more information about childhood cancer, please refer to Cancer Australia’s Children’s Cancer website. More data on childhood cancer in Australia can be found at the Cancer Council Queensland’s Australian Childhood Cancer Statistics Online website.

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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for acute lymphoblastic leukaemia, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for acute myeloid leukaemia, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for ependymoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for Ewing sarcoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for hepatoblastoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for Hodgkin lymphoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for medulloblastoma and other central nervous system embryonal tumours, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for neuroblastoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for non-Hodgkin lymphoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for non-rhabdomyosarcoma soft tissue sarcoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for osteosarcoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for ovarian tumours, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for retinoblastoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for rhabdomyosarcoma, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for testicular tumours, 2006-2015
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Notes

• Data are sourced from the Australian Childhood Cancer Register managed by the Cancer Council Queensland. More information about data sources can be found in the ‘About the Data’ tab.
• This analysis presents five-year relative survival estimates using the cohort method as outlined in the methodology. Five-year relative survival was determined by a follow-up on mortality status to 31 December 2016.
• Error bars indicate 95% confidence intervals.
• 'Tier 1' is a less-detailed staging system for registries with limited resources and 'Tier 2' is more-detailed staging system for well-resourced registries. The Tier 1 and Tier 2 categories for stage at diagnosis may differ across cancer types due to differences in classification systems. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
• For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).

Five-year relative survival for Wilms tumour, 2006-2015

About this measure

The lack of high quality national cancer staging data is an identified gap in Australia. Stage at diagnosis indicates the extent to which a cancer has spread when first diagnosed. It is an important prognostic factor for cancer outcomes. It also provides contextual information for interpreting cancer outcomes, including survival, at a population level.⁴

In Australia, stage at diagnosis for childhood cancers is not always routinely recorded in medical records and, for those where it is recorded, the staging system used is rarely documented¹. As part of the Australian Government’s Investing in Medical Research - Fighting Childhood Cancer initiative, Cancer Australia has collaborated with the Cancer Council Queensland (CCQ), the Australasian Association of Cancer Registries (AACR), and state and territory population-based cancer registries in developing nationally-standardised methodologies for recording childhood cancer stage at diagnosis for 16 major cancer types.

Childhood cancers are rare and generally differ from adult cancer in their biology, clinical classification and treatment. For adult cancers, there are well-established and universally- accepted systems for determining stage at diagnosis. However, the staging systems used for adult cancers are not adequate for staging many cancers that occur in children, and there has been no universally-accepted system for staging for most childhood cancer types.

The Toronto Paediatric Cancer Stage Guidelines (referred to as ‘the Guidelines’) are intended to facilitate recording of the best estimate of childhood cancer stage by population cancer registries for use in epidemiological analysis and reporting. Cancer Australia supported the CCQ in developing Business Rules for the recording of national childhood cancer stage at diagnosis for invasive tumours based on the Guidelines. The Guidelines and associated Business Rules have been endorsed by the Union for International Cancer Control (UICC) and the International Association of Cancer Registries (IACR)^{5, 6}.

For each of the 16 major childhood cancer types, the Guidelines recommend a tiered system as follows:

Tier 1: A less-detailed staging system for registries with limited resources; and
Tier 2: A more-detailed staging system for well-resourced registries. Tier 2 stage categories can be collapsed to the Tier 1 categories to enable comparisons of data from different registries.

Some cancer types have the same stage categories for both the Tier 1 and Tier 2 criteria. Where there are differences in the Tier 1 and Tier 2 stage categories, the results for these Tiers are presented separately in the text with common groupings of ‘limited’ and ‘advanced’ stage provided, where relevant. The categories for stage at diagnosis may differ across cancer types due to differences in classification systems determined under the Guidelines. More detailed information on the staging systems and Business Rules is available on the CCQ website which can be accessed through the ‘References’ tab. A summary of the disease-specific Tier 1 and Tier 2 staging categories for each of the childhood cancer types are listed in the table below:

Type of cancer	Tier 1 stage categories	Tier 2 stage categories	Stage categories used for this report *
Acute lymphoid leukaemia⁷	CNS-	CNS1	CNS-
Acute lymphoid leukaemia⁷	CNS+	CNS2, CNS3	CNS+
Acute myeloid leukaemia⁸	CNS-		CNS-
Acute myeloid leukaemia⁸	CNS+		CNS+
Ependymoma⁹	Localised	M0	Limited
Ependymoma⁹	Metastatic	M1, M2, M3, M4	Advanced
Ewing sarcoma¹⁰	Localised		Limited
Ewing sarcoma¹⁰	Metastatic		Advanced
Hepatoblastoma¹¹	Localised		Limited
Hepatoblastoma¹¹	Metastatic		Advanced
Hodgkin lymphoma¹²	Ann Arbor-stage IA/B		Limited
	Ann Arbor-stage IIA/B		Limited
	Ann Arbor-stage IIIA/B		Advanced
	Ann Arbor-stage IVA/B		Advanced
Medulloblastoma and other CNS embryonal tumours⁹	Localised	M0	Limited
Medulloblastoma and other CNS embryonal tumours⁹	Metastatic	M1, M2, M3, M4	Advanced
Neuroblastoma¹³	Localised	INRGSS-Localised L1	Limited
	Locoregional	INRGSS-locoregional L2	Limited
	Metastatic	INRGSS-metastatic M	Advanced
	INRGSS-MS disease	INRGSS-MS disease	Advanced
Non-Hodgkin lymphoma¹⁴	Limited	St Jude/Murphy-stage I, II, III	Limited
Non-Hodgkin lymphoma¹⁴	Advanced	St Jude/Murphy-stage IV	Advanced
Non-rhabdomyosarcoma soft tissue sarcoma¹⁰	Localised	TNM stage I, II, III	Limited
Non-rhabdomyosarcoma soft tissue sarcoma¹⁰	Metastatic	TNM stage IV	Advanced
Osteosarcoma¹⁰	Localised		Limited
Osteosarcoma¹⁰	Metastatic		Advanced
Ovarian germ cell tumours¹⁵	Localised	FIGO stage I	Limited
	Regional	FIGO stage II, III	Limited
	Metastatic	FIGO stage IV	Advanced
Retinoblastoma¹⁶	Localised	IRSS Stage 0, I, II	Limited
	Regional	IRSS Stage III	Limited
	Metastatic	IRSS Stage IV	Advanced
Rhabdomyosarcoma¹⁰	Localised	TNM stage I, II, III	Limited
Rhabdomyosarcoma¹⁰	Metastatic	TNM stage IV	Advanced
Testicular germ cell tumours¹⁰	Localised	TNM Stage I	Limited
	Regional	TNM Stage II	Limited
	Metastatic	TNM Stage III	Advanced
Wilms tumour^{17, 18}	Localised	Stage I/y-stage I, Stage II/y-stage II, Stage III/y-stage III	Limited
Wilms tumour^{17, 18}	Metastatic	Stage IV	Advanced

* Due to differences in the Tier 1 and Tier 2 stage categories for some cancer types, common groupings of ‘limited’ and ‘advanced’ stage are provided in some sections of this report. For this report, the stage categories classified as ‘Limited’ stage cancers are those confined to the site of origin or regional lymph nodes only, whereas ‘Advanced’ cancers are those that have spread to other parts of the body.

Current status

Survival from cancer is dependent on various factors, including: demographic characteristics, tumour biology, the stage of the cancer at diagnosis, other prognostic indicators, and treatment. Five-year relative survival, as used in this report, refers to the proportion of patients (expected to be) alive five years after the cancer diagnosis compared to survival for the general population matched by age, sex and calendar year.

Due to differences in the Tier 1 and Tier 2 stage categories for some cancer types, survival estimates for Tier 1 staging categories should not be compared to Tier 2 staging categories. Common groupings of ‘limited’ and ‘advanced’ stage are provided where relevant in the following sections. In this report:

‘Limited’ cancers are those confined to the site of origin or regional lymph nodes only;
‘Advanced’ cancers are those that have spread to other parts of the body.

Some cancer types have the same stage categories for both the Tier 1 and Tier 2 criteria. Where there are differences in the Tier 1 and Tier 2 stage categories, the results for these Tiers are presented separately in the descriptive text with common groupings of ‘limited’ and ‘advanced’ stage provided, where relevant. For some cancer types, stage categories were combined where necessary to obtain a sufficient number of cases to calculate survival estimates. To test whether differences in survival were statistically significant, p-values were derived using Poisson regression to model excess mortality by stage at diagnosis. Calculation of p-values excludes cases with “unknown” stage.

More detailed information on the classification of these cancer types and the staging systems used is available in the ‘About this measure’ tab and in the Business Rules which can be accessed through the ‘References’ tab.

Five-year relative survival by cancer type

For four of the 16 cancer types, five-year relative survival for advanced cancers was not examined due to small numbers:

Five-year relative survival was not examined for advanced stage ependymoma, retinoblastoma, testicular germ cell tumours and ovarian germ cell tumours.

For ten of the remaining 12 cancer types, five-year relative survival was higher if the cancer was diagnosed at a limited stage, or before the cancer had spread to other parts of the body (including CNS-):

For nine cancer types, children diagnosed at a limited stage had a significantly higher survival than those diagnosed at an advanced stage. These cancer types included acute lymphoblastic leukaemia, Ewing sarcoma, hepatoblastoma, medulloblastoma and other CNS embryonal tumours, neuroblastoma, non-rhabdomyosarcoma soft tissue sarcoma, osteosarcoma, rhabdomyosarcoma, and Wilms tumour.
For non-Hodgkin lymphoma, survival was higher for limited stage cancers (Tier 1 - limited: 91%; Tier 2 - stage I: 98%, stage II: 94%, stage III: 89%) compared to those with advanced stage cancers (Tier 1 - advanced: 85%; Tier 2 - stage IV: 85%), but these differences were not statistically significant.

For two of the 12 cancer types, five-year relative survival was similar regardless of stage at diagnosis:

There was no statistically significant difference in five-year relative survival by stage at diagnosis for acute myeloid leukaemia (77% for CNS-, and 76% for CNS+).
For children diagnosed with Hodgkin lymphoma, survival was high (at least 97%) regardless of stage at diagnosis.

Some cancer types had notably lower survival outcomes when the cancer was diagnosed at an advanced stage (metastatic). Five-year relative survival was lower than 50% for children diagnosed with advanced medulloblastoma (Tier 1 - metastatic: 42%; Tier 2 - M1/M2: 45%, M3: 40%), non-rhabdomyosarcoma soft tissue sarcoma (Tier 1 - metastatic: 34%; Tier 2 - stage IV: 34%), and osteosarcoma (metastatic: 36%).

Choose a cancer type below for further information:

Acute lymphoblastic leukaemia

For acute lymphoblastic leukaemia, stage at diagnosis categories are based on whether the central nervous system (CNS) is involved in the disease. CNS− refers to cancers where there is no central nervous system involvement (under Tier 2 the relevant category is CNS1) and CNS+ refers to those where there is central nervous system involvement (under Tier 2 the two relevant categories area: CNS2 and CNS3).

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with acute lymphoblastic leukaemia with no central nervous system involvement (CNS−,94%) compared to children diagnosed with central nervous system involvement (CNS+, 88%). (Tier 1 staging criteria).
Significantly higher for children diagnosed with acute lymphoblastic leukaemia with no central nervous system involvement (CNS1: 94%) compared to children diagnosed with central nervous system involvement at CNS2 (CNS2: 87%,) but not compared to children diagnosed at CNS3: 88%. (Tier 2 staging criteria).

Acute myeloid leukaemia

For acute myeloid leukaemia, the stage categories are based on whether the CNS is involved in the disease. The Tier 1 and Tier 2 staging criteria are the same for this cancer type. CNS- refers to cancers where there is no central nervous system involvement and CNS+ refers to when there is central nervous system involvement.

In the period 2006−2015, 5-year relative was:

Moderate for children diagnosed with acute myeloid leukaemia with no central nervous system involvement (CNS−, 77%) and children diagnosed with central nervous system involvement (CNS+, 76%).

Ependymoma

For ependymoma, stage at diagnosis categories are defined according to whether the tumour was localised (under Tier 2 the relevant category is M0) or metastatic (under Tier 2 the relevant categories are - M1, M2, M3, M4). For this cancer type, these Tier 2 categories provide further detail about the site of metastases.

In the period 2006−2015, 5-year relative survival was:

Moderate for children diagnosed with limited stage disease (Tier 1 - localised: 72%; Tier 2 - M0: 72%). There was an insufficient number of cases to calculate survival for children diagnosed with advanced stage disease (Tier 1 - metastatic; Tier 2 - M1, M2, M3, M4).

Ewing sarcoma

For Ewing sarcoma, the stage categories are defined according to whether the tumour was localised or metastatic. The Tier 1 and Tier 2 staging criteria are the same for this cancer type.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (localised: 88%) compared to children diagnosed with advanced stage disease (metastatic: 74%).

Hepatoblastoma

For hepatoblastoma, the stage categories are defined according to whether the tumour was localised or metastatic. The Tier 1 and Tier 2 staging criteria are the same for this cancer type.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (localised: 91%) compared to children diagnosed with advanced stage disease (metastatic: 68%).

Hodgkin lymphoma

For Hodgkin lymphoma, the stage categories are defined using the Ann Arbor-stage system12. This system classifies in order of increasing stage from limited to advanced stage disease as: IA, IB, IIA, IIB, IIIA, IIIB, IVA, and IVB. The suffixes ‘A’ and ‘B’ are used to denote the absence or presence of defined constitutional symptoms, respectively. The Tier 1 and Tier 2 staging criteria are the same for this cancer type.

In the period 2006−2015: 5-year relative survival was:

High (100%) for children diagnosed with stage IA/IB, IIA/IIB or IVA/IVB disease. Survival was slightly lower for children diagnosed at stage IIIA/IIIB disease (97%) but this difference was not statistically significant.

Medulloblastoma and other central nervous system (CNS) embryonal tumours

For medulloblastoma and other CNS embryonal tumours, stage at diagnosis categories are defined according to whether the tumour was localised (under Tier 2 the relevant category is M0) or metastatic (under Tier 2 the relevant categories are M1, M2, M3, M4). For this cancer type, the Tier 2 categories provide further detail about the site of metastases.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (Tier 1 - localised: 69%; Tier 2 - M0: 69%) compared to children diagnosed with advanced stage disease (Tier 1 - metastatic: 42%; Tier 2 - M1/M2: 45%, M3: 40%). There was an insufficient number of cases to calculate survival for M4 stage disease.

Neuroblastoma

For neuroblastoma, stage at diagnosis categories are defined as localised, locoregional or metastatic. Localised cancers (under Tier 2 the relevant category is L1) are those confined to the site of origin. Locoregional cancers (under Tier 2 the relevant category is - L2) are those that have spread to nearby lymph nodes or tissues. Metastatic cancers (under Tier 2 the relevant categories are M, MS) are those that have spread to different parts of the body. MS refers to metastatic cancer for children younger than 18 months old that were confined to the skin, liver, and/or bone marrow. The Tier 1 staging classifications for neuroblastoma are simplified proxies of the Tier 2 criteria that do not require assessment of image-defined risk factors from cross-sectional imaging.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (Tier 1 - localised: 97%, locoregional: 89%; Tier 2 - L1: 97%, L2: 89%) compared to children diagnosed with advanced stage disease, excluding stage MS (Tier 1 - metastatic: 62%; Tier 2 - M: 62%). Survival for children aged less than 18 months at diagnosis with stage MS neuroblastoma (Tier 1 and Tier 2 - MS: 94%) was similar to the survival for those with limited stage disease.

Non-Hodgkin lymphoma

For non-Hodgkin lymphoma, stage at diagnosis categories are defined as limited (under Tier 2 the relevant categories are Stage I, II, and III) or advanced stage (under Tier 2 the relevant category is stage IV). For this cancer type, limited cancers are those confined to the site of origin or regional lymph nodes only, whereas advanced cancers are those that have spread to the central nervous system or bone marrow.

In the period 2006−2015, 5-year relative survival was:

Slightly higher for children diagnosed with limited stage disease (Tier 1 - limited: 91%; Tier 2 - stage I: 98%, stage II: 94%, stage III: 89%) compared to children diagnosed with advanced stage disease (Tier 1 - advanced: 85%; Tier 2 - stage IV: 85%), but these differences were not statistically significant.

Non-rhabdomyosarcoma soft tissue sarcoma

For non-rhabdomyosarcoma soft tissue sarcoma, stage at diagnosis categories are defined according to whether the tumour was localised (under Tier 2 the relevant categories are stage I, II, and III) or metastatic (under Tier 2 the relevant category is stage IV). For this cancer type, the Tier 2 criteria are based on a combination of anatomic site, tumour size, lymph node involvement and metastatic status.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (Tier 1 - localised 84%; Tier 2 - stage I/II: 89%, stage III: 70%) compared to children diagnosed with advanced stage disease (Tier 1 - metastatic: 34%; Tier 2 - stage IV: 34%).

Osteosarcoma

For osteosarcoma, the stage categories are defined according to whether the tumour was localised or metastatic. The Tier 1 and Tier 2 staging criteria are the same for this cancer type.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (localised: 82%) compared to children diagnosed with advanced stage disease (metastatic: 36%).

Ovarian germ cell tumours

For ovarian germ cell tumours, stage at diagnosis categories are defined as localised, regional, or metastatic. Localised cancers (under Tier 2 the relevant category is stage I) are those confined to the site of origin. Regional cancers (under Tier 2 the relevant categories are stage II, III) are those that have spread to nearby lymph nodes or tissues. Metastatic cancers (under Tier 2 the relevant category is stage IV) are those that have spread to different parts of the body.

In the period 2006−2015, 5-year relative survival was:

High for children diagnosed with limited stage disease (Tier 1 - localised: 100%; regional: 95%; Tier 2 - stage I: 100%; stage II/III: 95%). There was an insufficient number of cases to calculate survival for children diagnosed with advanced stage disease (metastatic or stage IV).

Retinoblastoma

For retinoblastoma, stage at diagnosis categories are defined as localised, regional, or metastatic. Localised cancers (under Tier 2 the relevant categories are stage 0, I, II) are those confined to the site of origin. Regional cancers (under Tier 2 the relevant category is stage III) are those that have spread to nearby lymph nodes or tissues. Metastatic cancers (under Tier 2 the relevant category is stage IV) are those that have spread to different parts of the body. For this cancer type, the Tier 1 definitions for staging retinoblastoma are a simplified version of Tier 2.

In the period 2006−2015, 5-year relative survival was:

High (100%) for children diagnosed with localised disease. There was an insufficient number of cases to calculate survival for regional or metastatic cancers. (Tier 1 stage criteria)
High (100%) for children diagnosed with localised stage 0, and 99% for children diagnosed at localised stage I. There was an insufficient number of cases to calculate survival for regional (stage II, stage III), and metastatic (stage IV) cancers. (Tier 2 stage criteria)

Rhabdomyosarcoma

For rhabdomyosarcoma, stage at diagnosis categories are defined according to whether the tumour was localised (under Tier 2 the relevant categories are Stage I, II, and III) or metastatic (under Tier 2 the relevant category is Stage IV). For this cancer type, the Tier 2 criteria are based on a combination of anatomic site, tumour size, lymph node involvement and metastatic status.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (Tier 1 - localised: 81%; Tier 2 - stage I: 87%, stage II: 80%, stage III: 77%) compared to children diagnosed with advanced stage disease (Tier 1 - metastatic: 52%; Tier 2 - stage IV: 52%).

Testicular germ cell tumours

For testicular germ cell tumours, stage at diagnosis categories are defined according to whether the tumour was localised, regional or metastatic. Localised cancers (under Tier 2 the relevant category is Stage I) are those confined to the site of origin. Regional cancers (under Tier 2 the relevant category is Stage II) are those that have spread to nearby lymph nodes or tissues. Metastatic cancers (under Tier 2 the relevant category is Stage III) are those that have spread to different parts of the body. For this cancer type, the Tier 2 criteria are based on a combination of tumour size, lymph node involvement and metastatic status.

In the period 2006−2015, 5-year relative survival was:

High (100%) for children diagnosed with localised stage disease (Tier 2 - stage I). There was an insufficient number of cases to calculate survival for regional (Tier 2 - stage II) or metastatic stage disease (Tier 2 - stage III).

Wilms tumour

For Wilms tumour, stage at diagnosis categories are defined according to whether the tumour was localised (under Tier 2 the relevant categories are Stage I/yI, II/yII, and III/yIII) or metastatic (under Tier 2 the relevant category is Stage IV). For this cancer type, there are two staging systems used. The Children’s Oncology Group (COG)/National Wilms Tumour Study Group (NWTSG) staging system is used for children who have not received chemotherapy prior to surgery; and the International Society of Paediatric Oncology (SIOP) system is used for children who have received chemotherapy prior to surgery.17, 18 A prefix “y” is used to denote SIOP stage, except for metastatic stage which is denoted as “IV” in both systems.

In the period 2006−2015, 5-year relative survival was:

Significantly higher for children diagnosed with limited stage disease (Tier 1 - localised: 95%; Tier 2 - stage I/yI: 94%, stage II/yII: 92%, stage III/yIII: 99%) compared to those with advanced stage disease (Tier 1 - metastatic: 85%; Tier 2 - stage IV: 85%).

About the data

Methodology^{19, 20}:

Relative survival is a measure of survival for people diagnosed with cancer compared to survival for the general population. It is defined as a ratio of:

Observed survival (numerator): The proportion of people who are alive following a specified amount of time (e.g. 1 year, 3 years or 5 years) after diagnosis of cancer (observed survival). Observed survival is calculated from population-based cancer data.
Expected survival (denominator): The proportion of people in the general population who are alive over the same time interval (note: of equivalent age and sex to those with the cancer diagnosis). Expected survival is calculated from life tables for the entire Australian population.

For example, if 6 in 10 people with cancer are alive 5 years after their diagnosis (observed survival of 0.6) and 9 in 10 people of equivalent age and sex from the general population are alive after the same 5 years (expected survival of 0.9), then the relative survival of people with cancer would be 0.6 divided 0.9 which equates to 0.67. This means that individuals with cancer are 67% as likely to be alive for 5 years after their diagnosis compared with their counterparts in the general population.

Two main approaches are available for calculating survival estimates: the traditional cohort method, which follows the same group of patients over time; and the period method, which is based on different groups of patients within an “at risk” window of time. Although the period method tends to produce more up-to-date estimates, particularly for longer-term survival, the cohort method was deemed to be more suitable for the purposes of this analysis given that it describes the survival experience of a clearly defined group of children and is often easier to interpret. Children diagnosed by autopsy or reported by death certificate only were excluded from the survival analysis.

A suite of Stata programs developed by Paul Dickman from the Karolinska Institutet in Sweden was used to generate the survival estimates (see http://www.pauldickman.com/rsmodel/stata_colon/). ²¹ These programs use a life table (or actuarial) method for calculating relative survival.

To test whether differences in survival were statistically significant, p-values were derived using Poisson regression to model excess mortality by stage at diagnosis. Calculation of p-values excludes cases with “unknown” stage.

Scope:

Information is available on the stage at diagnosis and relative survival by stage for the following cancer types:

Type of cancer^(a)	Broad tissue of origin*	No. cases (2006–2015)	Proportion staged Tier 1 (%)	Proportion staged Tier 2 (%)
Acute lymphoblastic leukaemia	Blood and bone marrow	1,720	96%^(b)	95%
Acute myeloid leukaemia	Blood and bone marrow	314	83%	83%
Ependymoma^(c),(d)	Brain and central nervous system	121	93%	93%
Ewing sarcoma^(c)	Bone or soft tissues around bones	118	98%	98%
Hepatoblastoma^(c)	Liver	91	97%	97%
Hodgkin lymphoma	Lymphatic system/ lymphocytes	226	95%	95%
Medulloblastoma and other central nervous system (CNS) embryonal tumours	Brain and central nervous system	322	95%	95%
Neuroblastoma	Sympathetic nervous system	443	97%	97%
Non-Hodgkin lymphoma	Lymphatic system/ lymphocytes	341	96%	96%
Non-rhabdomyosarcoma soft tissue sarcoma	Soft tissues	158	92%^(b)	86%
Osteosarcoma^(c)	Bone or soft tissues around bones	104	95%	95%
Ovarian germ cell tumours ^(c),(d)	Ovary	45	98%	98%
Retinoblastoma^(c),(d)	Eye	178	98%^(b)	97%
Rhabdomyosarcoma	Soft tissues	205	96%^(b)	89%
Testicular germ cell tumours ^(c),(d)	Testis	33	97%	97%
Wilms tumour	Kidney	291	93%	93%
Total		4,710	95%	94%

*Groupings have been adapted from the International Classification of Childhood Cancers, 3rd edition (ICCC-3) Diagnostic Groups

Notes:

(a) Type of cancer classified according to the International Classification of Childhood Cancers, version 3 (ICCC-3)

(b) This proportion differs slightly from the proportion of cases staged using the more detailed Tier 2 staging criteria due to differences in the level of information required to assign stage at diagnosis.

(c) The proportion of cancers with ‘unknown’ stage at diagnosis should be interpreted with caution due to small numbers.

(d) The proportion of cases diagnosed as regional or metastatic should be interpreted with caution due to small numbers. Relative survival estimates for some stage categories could not to be calculated due to small numbers.

Toronto Paediatric Cancer Stage Guidelines and Business Rules for recording childhood cancer stage

The Business Rules are a set of detailed instructions which outline the data components required for defining each stage category for each of the 16 childhood cancer types childhood cancer types. Cancer Australia supported CCQ in developing these Business Rules which are based on Toronto Paediatric Cancer Stage Guidelines².

The Guidelines provide a framework for population-based cancer registries to record comparable and consistent information on childhood cancer stage at diagnosis from data sources available to them. Staging systems recommended in the Guidelines are not intended for clinical management. These data are intended for statistical purposes only where comparability and statistical completeness is the key. Specifically, the recording of childhood cancer stage using the Guidelines and associated Business Rules is intended to facilitate collection of comparable data internationally on childhood cancer stage by population cancer registries for use in epidemiological analysis and reporting¹.

The Guidelines and associated Business Rules have been endorsed by the Union for International Cancer Control (UICC) and the International Association of Cancer Registries (IACR)^{5, 6}. The Business Rules have also been accepted by the AACR as a standard for the collection of childhood cancer stage information in Australia. More detailed information on the Business Rules is available on the CCQ website which can be accessed through the ‘References’ tab.

Australian Childhood Cancer Register

The data reported in this measure for 2006–2015 are sourced from the Australian Childhood Cancer Registry (ACCR), which includes all childhood cancer cases diagnosed in Australia since 1983 for children aged 0 -14 years²². All Australian states and territories have legislation that makes cancer a notifiable disease. Various designated bodies, i.e., institutions such as hospitals, pathology laboratories and registries of births, deaths and marriages, are required to report cancer cases and deaths to their jurisdictional population-based cancer registries (PBCRs). With ethical approval, and consistent with their legislative authority, each jurisdictional PBCR provides information on all registered incident childhood cancer cases to the ACCR.

To assign stage at diagnosis using the Business Rules, detailed information from patient medical records was recorded by the ACCR clinical data manager with the necessary ethics and legislative approvals during site visits to the 10 major paediatric hospitals in Australia:

Hospital
Sydney Children’s Hospital, NSW	Queensland Children’s Hospital, QLD
New Children’s Hospital (Westmead), NSW	Mater Children’s Hospital, QLD
John Hunter Hospital, NSW	Perth Children’s Hospital, WA
Royal Children’s Hospital, VIC	Women’s and Children’s Hospital, SA
Monash Children’s Hospital, VIC	Royal Hobart Hospital, TAS

Stage at diagnosis was assigned electronically by algorithms developed from the Business Rules using data from the following sources:

Clinical case notes	Haematology reports
Diagnostic imaging	Cytology reports
Histology reports	Cerebrospinal fluid (CSF) reports

For this measure, five-year relative survival for children by stage at diagnosis is referred to as “survival”. Five-year relative survival was determined by a follow-up on mortality status to 31 Dec 2016.

Data caveats

This analysis presents five-year relative survival estimates using the cohort method for children aged 0 -14 years diagnosed with one of the 16 cancer types rounded up to the nearest whole number for the diagnostic years 2006−2015 inclusive.
Those diagnosed by autopsy or death certificate only were excluded from the survival analysis.
Error bars represent 95% confidence intervals.
To test whether differences in survival were statistically significant, p-values were derived using Poisson regression to model excess mortality by stage at diagnosis. Calculation of p-values excludes cases with “unknown” stage. Survival estimates for Tier 1 staging categories should not be compared to Tier 2 staging categories. Cases with “unknown” stage have been excluded from calculations to determine statistical significance.
Five-year relative survival was determined by a follow-up on mortality status to 31 Dec 2016.
These data are aggregated across multiple years (2006–2015) due to small numbers. Using the more detailed Tier 2 criteria, stage could be assigned to 4,418 of the 4,710 childhood cancer cases (94%) that were eligible for staging. Cancer cases not eligible for staging included those with an ineligible morphology for staging using the Business Rules, children who were not Australian residents, or those whose source medical records were not located at one of the in-scope hospitals.
For some cancer types there was an insufficient number of cases to calculate a survival estimate for some stage categories (including “unknown” stage).
Some cancer types have the same stage categories for both the Tier 1 and Tier 2 criteria. Where there are differences in the Tier 1 and Tier 2 stage categories, the respective results are presented separately in the text with common groupings of ‘limited’ and ‘advanced’ stage provided, where relevant.
Collection of these data has provided an insight into differences in the availability, extent and accessibility of information that is required to derive stage across PBCRs for childhood cancers. The Business Rules allow for a basic stage at diagnosis to be assigned in lower resource registries (Tier 1) and a more detailed stage system to be used by higher resource registries (Tier 2).

References

Activity in this area

Australian Government Department of Health, 2017 - Investing in Medical Research – fighting childhood cancer. (http://www.health.gov.au/internet/budget/publishing.nsf/Content/budget2017-factsheet52.htm)

Cancer Australia – Children’s Cancer website (https://childrenscancer.canceraustralia.gov.au/)

Cancer Council Queensland – The Australian Childhood Cancer Registry (ACCR) (https://cancerqld.org.au/research/cancer-registries/australian-childhood-cancer-registry/).

Cancer Australia, 2015 - Children’s Cancer website (https://childrenscancer.canceraustralia.gov.au/about-childrens-cancer)

Cancer Australia, 2008 - A National Cancer Data Strategy for Australia. (https://canceraustralia.gov.au/sites/default/files/publications/ncds_final_web1_504af02093a68.pdf).

Data

Detailed results from the collection of childhood cancer stage data as part of this project are available in the following journal articles:

Youlden, D.R., Gupta, S., Frazier, A.L., Moore, A.S., Baade, P.D., Valery, P.C., Green, A.C., and Aitken, J.F. 2019. Stage at diagnosis for children with blood cancers in Australia: Application of the Toronto Paediatric Cancer Stage Guidelines in a population-based national childhood cancer registry. Pediatr Blood Cancer, 66: p. e27683.
Youlden, D.R., Frazier, A.L., Gupta, S., Pritchard-Jones, K., Kirby, M.L., Baade, P.D., Green, A.C., Valery, P.C., and Aitken, J.F. 2019. Stage at diagnosis for childhood solid cancers in Australia: A population-based study. Cancer Epidemiol, 59: p. 208-214.

Australian Childhood Cancer Registry, Cancer Council Queensland. Australian Childhood Cancer Statistics Online. CCQ: Brisbane, Australia; 2018. Based on data from the ACCR (1983-2015). (https://cancerqld.org.au/research/queensland-cancer-statistics/accr/)

Australian Institute of Health and Welfare 2019. Australian Cancer Incidence and Mortality (ACIM) books provide incidence and mortality by cancer type and selected demographic groups. (https://www.aihw.gov.au/reports/cancer/acim-books/contents/acim-books).

Australian Institute of Health and Welfare 2011. Cancer in adolescents and young adults in Australia. Cancer series no 62. Cat. no. CAN 59. Canberra: AIHW. (https://www.aihw.gov.au/reports/cancer/cancer-in-adolescents-and-young-adults-in-australi/contents/table-of-contents)

Business rules³ for assigning childhood cancer stage at diagnosis are accessible at: (https://cancerqld.blob.core.windows.net/content/docs/childhood-cancer-staging-for-population-registries.pdf)

References

1. Aitken, J.F., Youlden, D.R., Moore, A.S., Baade, P.D., Ward, L.J., Thursfield, V.J., Valery, P.C., Green, A.C., Gupta, S., and Frazier, L.A. 2018. Assessing the feasibility and validity of the Toronto Childhood Cancer Stage Guidelines: a population-based registry study. The Lancet Child & Adolescent Health, 2(3): p. 173-179.

2. Gupta, S., Aitken, J.F., Bartels, U., Brierley, J., Dolendo, M., Friedrich, P., Fuentes-Alabi, S., Garrido, C.P., Gatta, G., Gospodarowicz, M., Gross, T., Howard, S.C., Molyneux, E., Moreno, F., Pole, J.D., Pritchard-Jones, K., Ramirez, O., Ries, L.A.G., Rodriguez-Galindo, C., Shin, H.Y., Steliarova-Foucher, E., Sung, L., Supriyadi, E., Swaminathan, R., Torode, J., Vora, T., Kutluk, T., and Frazier, A.L. 2016. Paediatric cancer stage in population-based cancer registries: the Toronto consensus principles and guidelines. Lancet Oncol, 17(4): p. e163-e172.

3. Aitken, J.F., Youlden, D.R., Moore, A.S., Baade, P.D., Ward, L.J., Thursfield, V.J., Valery, P.C., Green, A.C., Gupta, S., and Frazier, A.L. 2017. Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines. Brisbane, Australia: Cancer Council Queensland and Cancer Australia.

4. Australian Institute of Health and Welfare 2017. Cancer in Australia 2017. Cat. no. CAN 100. AIHW: Canberra.

5. Brierley, J., Gospodarowicz, M., and Wittekind, C. 2016. The TNM Classification of Malignant Tumours, 8th edition. . Lyon, France: Union for International Cancer Control (UICC).

6. International Association of Cancer Registries 2019. Paediatric Cancer Stage Guidelines - The IACR endorses the published Toronto Childhood Cancer Stage Guidelines and associated staging rules for use by population-based cancer registries.; Available from: <http://www.iacr.com.fr/index.php?option=com_content&view=article&id=153&Itemid=657>.

7. Winick, N., Devidas, M., Chen, S., Maloney, K., Larsen, E., Mattano, L., Borowitz, M.J., Carroll, A., Gastier-Foster, J.M., Heerema, N.A., Willman, C., Wood, B., Loh, M.L., Raetz, E., Hunger, S.P., and Carroll, W.L. 2017. Impact of Initial CSF Findings on Outcome Among Patients With National Cancer Institute Standard- and High-Risk B-Cell Acute Lymphoblastic Leukemia: A Report From the Children's Oncology Group. J Clin Oncol, 35(22): p. 2527-2534.

8. Abbott, B.L., Rubnitz, J.E., Tong, X., Srivastava, D.K., Pui, C.H., Ribeiro, R.C., and Razzouk, B.I. 2003. Clinical significance of central nervous system involvement at diagnosis of pediatric acute myeloid leukemia: a single institution's experience. Leukemia, 17(11): p. 2090-2096.

9. Harisiadis, L. and Chang, C.H. 1977. Medulloblastoma in children: a correlation between staging and results of treatment. Int J Radiat Oncol Biol Phys, 2(9-10): p. 833-841.

10. Brierley, J., Gospodarowicz, M., and Wittekind, C. 2016. The TNM Classification of Malignant Tumours, 8th edition. Lyon, France: Union for International Cancer Control (UICC).

11. Walters, S., Maringe, C., Butler, J., Brierley, J.D., Rachet, B., and Coleman, M.P. 2013. Comparability of stage data in cancer registries in six countries: lessons from the International Cancer Benchmarking Partnership. Int J Cancer, 132(3): p. 676-685.

12. Carbone, P.P., Kaplan, H.S., Musshoff, K., Smithers, D.W., and Tubiana, M. 1971. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res, 31(11): p. 1860-1861.

13. Monclair, T., Brodeur, G.M., Ambros, P.F., Brisse, H.J., Cecchetto, G., Holmes, K., Kaneko, M., London, W.B., Matthay, K.K., Nuchtern, J.G., von Schweinitz, D., Simon, T., Cohn, S.L., Pearson, A.D., and Force, I.T. 2009. The International Neuroblastoma Risk Group (INRG) staging system: an INRG Task Force report. J Clin Oncol, 27(2): p. 298-303.

14. Murphy, S.B. 1980. Classification, staging and end results of treatment of childhood non-Hodgkin's lymphomas: dissimilarities from lymphomas in adults. Semin Oncol, 7(3): p. 332-339.

15. Prat, J. and Oncology, F.C.o.G. 2014. Staging classification for cancer of the ovary, fallopian tube, and peritoneum. Int J Gynaecol Obstet, 124(1): p. 1-5.

16. Chantada, G., Doz, F., Antoneli, C.B., Grundy, R., Clare Stannard, F.F., Dunkel, I.J., Grabowski, E., Leal-Leal, C., Rodriguez-Galindo, C., Schvartzman, E., Popovic, M.B., Kremens, B., Meadows, A.T., and Zucker, J.M. 2006. A proposal for an international retinoblastoma staging system. Pediatr Blood Cancer, 47(6): p. 801-805.

17. Orkin, S., Fisher, D., Look, A., Lux, S., D., G., and Nathan, D. 2009. Oncology of Infancy and Childhood. Philadelphia, PA: Saunders Elsevier.

18. Metzger, M.L. and Dome, J.S. 2005. Current therapy for Wilms' tumor. Oncologist, 10(10): p. 815-826.

19. Australian Childhood Cancer Registry, C.C.Q. 2018. ACCR Data Sources and Methodology. [Accessed 2018 29 May]; Available from: <https://cancerqld.org.au/research/queensland-cancer-statistics/accr-data-sources-methdology/>.

20. Australian Institute of Health and Welfare 2019. Cancer in Australia 2019. Cat. no. CAN 123. AIHW: Canberra.

21. Dickman, P. and Coviello, E. 2015. Estimating and modelling relative survival. The Stata Journal, 15(1): p. 186-215.

22. Baade, P.D., Youlden, D.R., Valery, P.C., Hassall, T., Ward, L., Green, A.C., and Aitken, J.F. 2010. Trends in incidence of childhood cancer in Australia, 1983-2006. Br J Cancer, 102(3): p. 620-626.

Summary

For nine cancer types, five-year relative survival was higher if the cancer was diagnosed at a limited stage, or before the cancer had spread to other parts of the body (including CNS-)For acute lymphoblastic leukaemia, Ewing sarcoma, hepatoblastoma, medulloblastoma and other CNS embryonal tumours, neuroblastoma, non-rhabdomyosarcoma soft tissue sarcoma, osteosarcoma, rhabdomyosarcoma, and Wilms tumour, children diagnosed at a limited stage had statistically significant higher survival than those diagnosed at an advanced stage. 
For non-Hodgkin lymphoma, survival was higher for children with limited (91%) compared to advanced stage disease (85%), but these differences were not statistically significant.

Five-year relative survival was similar regardless of stage at diagnosis for acute myeloid leukaemiaThere was no statistically significant difference in five-year relative survival by stage at diagnosis for acute myeloid leukaemia (77% for CNS-, and 76% for CNS+).

Five-year relative survival was high for Hodgkin lymphoma regardless of stage at diagnosisFor children diagnosed with Hodgkin lymphoma, survival was high (at least 97%) regardless of stage at diagnosis. 

Some cancer types had notably lower survival outcomes when the cancer was diagnosed at an advanced stage (metastatic)Five-year relative survival was lower than 50% for children diagnosed with advanced medulloblastoma and other CNS embryonal tumours (metastatic: 42%; M1/M2: 45%, M3: 40%), non-rhabdomyosarcoma soft tissue sarcoma (metastatic or stage IV: 34%), and osteosarcoma (metastatic: 36%). 

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Data

Five-year relative survival by cancer type

Summary

For nine cancer types, five-year relative survival was higher if the cancer was diagnosed at a limited stage, or before the cancer had spread to other parts of the body (including CNS-)

Five-year relative survival was similar regardless of stage at diagnosis for acute myeloid leukaemia

Five-year relative survival was high for Hodgkin lymphoma regardless of stage at diagnosis

Some cancer types had notably lower survival outcomes when the cancer was diagnosed at an advanced stage (metastatic)

Related features

Childhood cancer stage at diagnosis and survival

Relative survival by stage at diagnosis 2011–2016, a snapshot in time

National cancer stage at diagnosis data