Published 06 Oct, 2021

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

For this measure, the proportion of incident cancer cases in each stage category is also referred to as the “stage distribution”. The 'distribution of childhood cancer stage' measure is one of four measures reported on the National Cancer Control Indicators (NCCI) website that are related to childhood cancers. The other measures are:

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. Additional data on childhood cancer in Australia can be found at the Cancer Council Queensland’s Australian Childhood Cancer Statistics Online website.

 

    Charts
    • 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.
      • The analysis for staging completeness presents unadjusted crude proportions of cancer cases for which stage data were available.
      • The analysis for proportional distribution is based on a total of 7,172 cases diagnosed in 2006-2015 for all childhood cancer types. Of these, there were 5,074 cancer cases diagnosed for the sixteen major cancer types.
      • '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, the proportion of cases that had an 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Proportion of all childhood cancer cases diagnosed and staging completeness, by cancer type, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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. Common groupings of ‘limited’ and ‘advanced’ stage are provided in this chart, where relevant. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
      • For some cancer types, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution by cancer type, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for acute lymphoblastic leukaemia, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for acute myeloid leukaemia, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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. Common groupings of ‘limited’ and ‘advanced’ stage are provided in this chart, where relevant. More information on the Tier 1 and Tier 2 staging categories can be found in the ‘About this measure’ tab.
      • For some cancer types, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for ependymoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for Ewing sarcoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for hepatoblastoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for Hodgkin lymphoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for medulloblastoma and other central nervous system embryonal tumours, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for neuroblastoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution non-Hodgkin lymphoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for non-rhabdomyosarcoma soft tissue sarcoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for osteosarcoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for ovarian tumours, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for retinoblastoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution rhabdomyosarcoma, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for testicular tumours, 2006-2015
    • 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 unadjusted crude proportions of cancer cases for which stage data were available.
      • '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, the proportion of cases that had an 'advanced' or 'unknown' stage at diagnosis should be interpreted with caution due to small numbers. More information on the scope of these data can be found in the ‘About the Data’ tab.
      Table caption
      Childhood cancer stage distribution for Wilms tumour, 2006-2015

    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, 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-

    CNS+

    CNS2, CNS3

    CNS+

    Acute myeloid leukaemia8

    CNS-

    CNS-

    CNS+

    CNS+

    Ependymoma9

    Localised

    M0

    Limited

    Metastatic

    M1, M2, M3, M4

    Advanced

    Ewing sarcoma10

    Localised

    Limited

    Metastatic

    Advanced

    Hepatoblastoma11

    Localised

    Limited

    Metastatic

    Advanced

    Hodgkin lymphoma12

    Ann Arbor-stage IA/B

    Limited

    Ann Arbor-stage IIA/B

    Ann Arbor-stage IIIA/B

    Advanced

    Ann Arbor-stage IVA/B

    Medulloblastoma and other CNS embryonal tumours9

    Localised

    M0

    Limited

    Metastatic

    M1, M2, M3, M4

    Advanced

    Neuroblastoma13

    Localised

    INRGSS-Localised L1

    Limited

    Locoregional

    INRGSS-locoregional L2

    Metastatic

    INRGSS-metastatic M

    Advanced

    INRGSS-MS disease

    INRGSS-MS disease

    Non-Hodgkin lymphoma14

    Limited

    St Jude/Murphy-stage I, II, III

    Limited

    Advanced

    St Jude/Murphy-stage IV

    Advanced

    Non-rhabdomyosarcoma soft tissue sarcoma10

    Localised

    TNM stage I, II, III

    Limited

    Metastatic

    TNM stage IV

    Advanced

    Osteosarcoma10

    Localised

    Limited

    Metastatic

    Advanced

    Ovarian cancer15

    Localised

    FIGO stage I

    Limited

    Regional

    FIGO stage II, III

    Metastatic

    FIGO stage IV

    Advanced

    Retinoblastoma16

    Localised

    IRSS Stage 0, I, II

    Limited

    Regional

    IRSS Stage III

    Metastatic

    IRSS Stage IV

    Advanced

    Rhabdomyosarcoma10

    Localised

    TNM stage I, II, III

    Limited

    Metastatic

    TNM stage IV

    Advanced

     

    Testicular germ cell tumours10

    Localised

    TNM Stage I

    Limited

    Regional

    TNM Stage II

    Metastatic

    TNM Stage III

    Advanced

    Wilms tumour17, 18

    Localised

    Stage I/y-stage I, Stage II/y-stage II, Stage III/y-stage III

    Limited

    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.

     

    The distribution of cancer stage at diagnosis is used to indicate if a cancer is generally diagnosed at a limited or advanced stage. 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 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, the proportion of cases staged using the Tier 1 criteria may differ from the Tier 2 criteria due to differences in the level of information required to assign stage at diagnosis.

    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.

    Proportional distribution and staging completeness for childhood cancers

    Together, the 16 major childhood cancer types accounted for 71% of all childhood cancer cases diagnosed in 2006−2015. Of these cancer types, the most commonly diagnosed was acute lymphoblastic leukaemia, representing 25% of all childhood cancer cases diagnosed in 2006−2014. Neuroblastoma represented 7% and Hodgkin lymphoma represented 5% of all cases diagnosed in this period. The other 13 cancer types each accounted for less than 5% of all childhood cancer cases diagnosed in 2006−2015

    The proportions of cases that were able to be staged among the 16 major childhood cancer types using the more detailed Tier 2 criteria were high overall, although higher again using the less detailed Tier 1 criteria for some cancer types. In the period 2006−2015, a high proportion of cases were staged overall using the Tier 1 (95%) or Tier 2 staging criteria (94%). The proportion of cases staged for each cancer type using the Tier 1 criteria ranged from 83% (for acute myeloid leukaemia) to 98% (for retinoblastoma, ovarian germ cell tumours and Ewing sarcoma). Using the more detailed Tier 2 criteria, the proportion of cases staged ranged from 83% (for acute myeloid leukaemia) to 98% (for ovarian germ cell tumours and Ewing sarcoma).

    Stage distribution by cancer type

    For most of the 16 cancer types, the majority of cases (>50%) diagnosed in 2006−2015 were diagnosed at a ‘limited’ stage:

    • For 13 cancer types the majority of cases were diagnosed at a ‘limited’ stage before the cancer had spread to other parts of the body. Using the Tier 1 criteria, this ranged from 64% of cases (for hepatoblastoma) to 97% of cases for retinoblastoma.
    • Using the more detailed Tier 2 criteria, this ranged from 64% of cases for hepatoblastoma to 97% of cases for retinoblastoma.
    • For Hodgkin lymphoma and neuroblastoma, a relatively high proportion of cases were diagnosed at an ‘advanced’ stage:
      • Hodgkin lymphoma − IIIA/B: 19%, IVA/B: 27%; and
      • neuroblastoma − metastatic or M: 48%, MS: 7%.
    • For acute lymphoblastic leukaemia and acute myeloid leukaemia, stage at diagnosis is determined by central nervous system (CNS) involvement. Most children diagnosed with acute lymphoblastic leukaemia (86%) or acute myeloid leukaemia (55%) were classified as having no central nervous system involvement (CNS-)

    Acute lymphoblastic leukaemia

    For acute lymphoblastic leukaemia, the stage 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 are: CNS2 and CNS3).

    In the period 2006−2015:

    • A high proportion of acute lymphoblastic leukaemia cases were able to be staged (96% using Tier 1 and 95% using Tier 2 criteria).
    • A high proportion of cases (86%) had no central nervous system involvement (Tier 1 CNS−; Tier 2 - CNS1).

    • Less than 10% of cases had central nervous system involvement (Tier 1 - CNS+: 9%; Tier 2 - CNS2: 6%, CNS3: 2%).

     

    Acute myeloid leukaemia

    For acute myeloid leukaemia, stage at diagnosis 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:

    • A high proportion of acute myeloid leukaemia cases (83%) were able to be staged.
    • The majority of cases (55%) had no central nervous system involvement (CNS−).
    • A smaller proportion of cases (28%) had central nervous system involvement (CNS+).

     

    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:

    • A very high proportion of ependymoma cases (93%) were able to be staged using Tier 1 or Tier 2 criteria.
    • A high proportion of cases (84%) were diagnosed at limited stage (Tier 1 - localised; Tier 2 - M0).
    • Less than 10% of cases were diagnosed at advanced stage (Tier 1 - metastatic: 9%; Tier 2 - M1: 0%, M2: 4%, M3: 5%, M4: 0%).

     

    Ewing sarcoma

    For Ewing sarcoma, stage at diagnosis 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:

    • A very high proportion of Ewing sarcoma cases (97%) were able to be staged.
    • Almost two-thirds of cases (65%) were diagnosed at limited stage (localised).
    • Almost one-third of cases (32%) were diagnosed at advanced stage (metastatic).

     

    Hepatoblastoma

    For hepatoblastoma, stage at diagnosis 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:

    • A very high proportion of hepatoblastoma cases (97%) were able to be staged.
    • Almost two-thirds of cases (64%) were diagnosed at limited stage (localised).
    • One-third of cases (33%) were diagnosed at advanced stage (metastatic).

     

    Hodgkin lymphoma

    For Hodgkin lymphoma, stage at diagnosis categories are defined using the Ann Arbor-stage system.12 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:

    • A very high proportion of Hodgkin lymphoma cases (95%) were able to be staged.
    • Overall, the most common stage at diagnosis was IIA/IIB (33%) followed by IVA/IVB (26%), IIIA/IIIB (19%), and IA/IB (17%).
    • More than two-thirds of cases (68%) had no systemic symptoms (stage suffix ‘A’), whereas 27% had systemic symptoms recorded (stage suffix ‘B’). In particular, 22% of children with no systemic symptoms were stage IVA, whereas 42% of children with systemic symptoms were stage IVB.

     

    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:

    • A very high proportion of medulloblastoma and other CNS embryonal tumours (95%) were able to be staged using Tier 1 or Tier 2 criteria.
    • Almost two-thirds of cases (66%) were diagnosed at limited stage (Tier 1 - localised; Tier 2 - M0).
    • Less than one-third of cases were diagnosed at advanced stage (Tier 1 - metastatic: 29%; Tier 2 - M1: 2%, M2: 6%, M3: 21%, M4: 0%).

     

    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:

    • A very high proportion of neuroblastoma cases (97%) were able to be staged using Tier 1 or Tier 2 criteria.
    • Around two in five cases (42%) were diagnosed at limited stage (Tier 1 - localised: 25%, locoregional: 17%; Tier 2 - L1: 25%, L2: 17%).
    • The majority of cases (55%) were diagnosed at advanced stage (Tier 1 - metastatic: 48%, MS: 7%; Tier 2 - M: 48%, MS: 7%). Of the 77 children aged younger than 18 months at diagnosis who had metastatic disease, 64% were classified as stage M and 36% as stage MS.

     

    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:

    • A very high proportion of non-Hodgkin lymphoma cases (96%) were able to be staged using Tier 1 or Tier 2 criteria.
    • Around three-quarters of cases were diagnosed at limited stage (Tier 1 - limited: 74%; Tier 2 - stage I: 12%, stage II: 10%, stage III: 53%).
    • Less than one-quarter of cases (22%) were diagnosed at advanced stage (Tier 1 - advanced; Tier 2 - stage IV).

     

    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:

    • A high proportion of cases were able to be staged (Tier 1:92%; Tier 2:86%).
    • The majority of cases were diagnosed at limited stage (Tier 1 - localised: 73%; Tier 2 - stage I: 41%, stage II: 9%, stage III: 17%).
    • Less than one-fifth of cases were diagnosed at advanced stage (Tier 1 - metastatic 19%; Tier 2 - stage IV: 19%).

     

    Osteosarcoma

    For osteosarcoma, stage at diagnosis 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:

    • A very high proportion of osteosarcoma cases (95%) were able to be staged.
    • Almost three-quarters of cases (71%) were diagnosed at limited stage (localised).
    • Less than one-quarter of cases (24%) were diagnosed at advanced stage (metastatic).

     

    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:

    • A very high proportion of ovarian germ cell tumours (98%) were able to be staged using Tier 1 or Tier 2 criteria.
    • The majority of cases were diagnosed at limited stage (Tier 1 - localised: 49%, regional: 44%; Tier 2 - stage I: 49%, stage II: 13%, stage III: 31%).
    • A small proportion of cases were diagnosed at advanced stage (Tier 1 - metastatic: 4%; Tier 2 - stage IV: 4%).

     

    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:

    • A very high proportion of retinoblastoma cases were able to be staged (Tier 1: 98%; Tier 2: 97%).
    • A very high proportion of  cases were diagnosed at limited stage (Tier 1 - localised: 96%, regional: 1%; Tier 2 - stage 0: 30%, stage I: 65%, stage II: 1%).
    • A very small proportion of cases were diagnosed at advanced stage (Tier 1 - metastatic 1%; Tier 2 - stage IV: 1%).

     

     

    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:

    • A very high proportion of rhabdomyosarcoma cases were able to be staged (Tier 1: 96%; Tier 2: 89%).
    • The majority of cases were diagnosed at limited stage (Tier 1 - localised: 76%; Tier 2 - stage I: 29%, stage II: 12%, stage III: 28%).
    • Approximately one-fifth of cases were diagnosed at advanced stage (Tier 1 - metastatic: 21%; Tier 2 - stage IV: 21%)

     

    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:

    • A very high proportion of testicular germ cell tumours (97%) were able to be staged using Tier 1 or Tier 2 criteria.
    • A high proportion of cases (85%) were diagnosed at limited stage (Tier 1 - localised: 79%; regional: 6%; Tier 2 - stage I: 79%; stage II: 6%).
    • A small proportion of cases were diagnosed at advanced stage (Tier 1 - metastatic: 12%; Tier 2 - stage III: 12%).

     

    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:

    • A high proportion of cases (93%) were able to be staged for Wilms tumour using Tier 1 or Tier 2 criteria.
    • Around three-quarters of cases were diagnosed at limited stage (Tier 1 - localised: 77%; Tier 2 - stage I/yI: 28%, II/yII: 21%, stage III/yIII: 29%).
    • A smaller proportion of cases were diagnosed at advanced stage (Tier 1 - metastatic: 16%; Tier 2 - stage IV: 16%).

    Unit of analysis:

    The unadjusted crude proportion of childhood cancer cases (children aged 0 -14 years) for which stage data are available for 16 major childhood cancer types. Proportions are rounded to the nearest whole number in the descriptive text. More detailed information on the classification of these cancer types and the staging systems used is available in the Business Rules which can be accessed through the ‘References’ tab.

    Numerator: The number of incident cancer cases for which stage could be derived in a selected Tier 1 or Tier 2 childhood cancer stage at diagnosis category for a selected childhood cancer type.

    Denominator: All eligible records for the relevant childhood cancer type. The denominator includes cases with an "unknown" stage at diagnosis for which the registry did not have sufficient information to derive 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. .
     

    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 years19. 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 10 the 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
     

    Data caveats

    • This analysis presents crude proportions (i.e. they have not been adjusted) of cancers staged for children aged 0 -14 years rounded to the nearest whole number for the diagnostic years 2006−2015 inclusive.
    • 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.
    • 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).

    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, 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. 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

    Staging completeness was high overall for each of the 16 major childhood cancers

    The proportion of cases that could be staged for each of the 16 major childhood cancer types ranged from 83% to 98%.

    A high proportion of cases were diagnosed at a limited stage for most of the major childhood cancer types

    For 14 cancer types stage can be categorised as ‘limited’ or ‘advanced’, and for 12 of these cancer types the majority of cases were diagnosed at a ‘limited’ stage before the cancer had spread to other parts of the body, ranging from 64% for hepatoblastoma to 97% for retinoblastoma.

    A relatively high proportion of neuroblastoma cases were diagnosed at an advanced stage

    Advanced stage cancers accounted for 45% of cases for Hodgkin lymphoma and 55% of cases for neuroblastoma.

    For childhood leukaemias, the majority of cases had no central nervous system involvement

    Most children diagnosed with acute lymphoblastic leukaemia (86%) or acute myeloid leukaemia (55%) were classified as having no central nervous system involvement (CNS– ).