Childhood Cancer Staging Rules for Population Based Registries

Transcription

1 Childhood Cancer Staging Rules for Population Based Registries Based on the Toronto Paediatric Cancer Stage Guidelines 1

2 Suggested citation Aitken JF, Youlden DR, Ward LJ, Thursfield VJ, Baade PD, Hallahan AR, Green AC, Valery PC, Gupta S, Frazier AL, Childhood cancer staging rules for population registries, based on the Toronto Paediatric Cancer Stage Guidelines. Cancer Council Queensland: Brisbane, Australia. Acknowledgements This manual has been adapted from the work of the Australian Childhood Cancer Registry (Cancer Council Queensland). 1

3 Table of Contents Suggested citation... 1 Acknowledgements... 1 Table of Contents... 2 List of tables... 2 List of figures... 2 Abbreviations... 3 Introduction Acute lymphoblastic leukaemia Acute myeloid leukaemia Non-Hodgkin lymphoma Neuroblastoma Wilms tumour Non-rhabdomyosarcoma soft-tissue sarcoma Osteosarcoma Ewing sarcoma Retinoblastoma Hepatoblastoma Testicular cancer Ovarian cancer Medulloblastoma and other CNS embryonal tumours References List of tables Table 1: The Toronto Paediatric Cancer Stage Guidelines 6 Table 2: Paediatric Cancer Stage: TNM Equivalents 7 List of figures Figure 1a: Lymphatic regions above the diaphragm for the staging of Hodgkin s and Non-Hodgkin s lymphoma 13 Figure 1b: Lymphatic regions below the diaphragm for the staging of Hodgkin s and Non-Hodgkin s lymphoma 14 2

4 Abbreviations AJCC APCR COG CNS CSF FIGO ICCC-3 ICD-O-3 IDRF INRGSS IRSS M MIBG MRI MS MYCN NOS NWTSG PNET ppnet RBC RMS SEER SIOP STaR TNM UICC WBC WHO American Joint Committee on Cancer Australian Paediatric Cancer Registry Children s Oncology Group Central nervous system Cerebrospinal fluid International Federation of Gynaecological Oncologists International Classification of Childhood Cancer, Third Edition International Classification of Diseases for Oncology Third Edition Image-defined risk factors International Neuroblastoma Risk Group Staging System International Retinoblastoma Staging System Medulloblastoma Iodine-123 metaiodobenzylguanidine Magnetic resonance imaging special v-myc avian myelocytomatosis viral oncogene neuroblastoma derived homolog Not otherwise specified National Wilms tumour Study Group Primitive neuroectodermal tumour Peripheral neuroectodermal tumour Red blood cell count Rhabdomyosarcoma Surveillance, Epidemiology and End Results Programme International Society of Paediatric Oncology Staging, Treatment and Recurrence project Tumour, lymph nodes, metastasis Union for International Cancer Control White blood cell count World Health Organisation 3

5 Introduction The collection by population-based cancer registries of internationally consistent information on childhood cancer stage is essential for epidemiologic analysis, international benchmarking and meaningful comparisons of childhood cancer incidence and outcomes. The TNM system is the standard staging system for most adult cancers however it is inadequate for documenting extent of disease in children. Disease-specific staging systems have been developed for childhood cancers but, for many diagnostic groups, two or more systems are in clinical use and there is no internationally uniform standard suitable for population-based cancer registration.(1) The Toronto Paediatric Cancer Stage Guidelines for population cancer registries A consensus meeting was convened in 2014 by the Union for International Cancer Control (UICC), the Dana- Farber Cancer Institute and the Hospital for Sick Children, Toronto to address the lack of consistent information on childhood cancer stage in population registries. For each of a subset of major ICCC-3 diagnostic groups/subgroups, the meeting reviewed all disease-specific paediatric cancer staging systems currently in use and recommended the one most suitable for use by population-based cancer registries. The recommended staging systems are listed in the Toronto Paediatric Cancer Stage Guidelines (1). The Guidelines recommend disease-specific staging systems for acute lymphoblastic leukaemia, acute myeloid leukaemia, Hodgkin s lymphoma, non-hodgkin lymphoma, neuroblastoma, Wilms tumour, rhabdomyosarcoma, non-rhabdomyosarcoma soft-tissue sarcomas, osteosarcoma, Ewing sarcoma, retinoblastoma, hepatoblastoma, germ cell tumours (testicular cancer and ovarian cancer), medulloblastoma and ependymoma. These 16 groups encompass approximately 75% of all childhood cancers. Here we provide detailed criteria for the staging systems recommended in the Guidelines. These criteria may be used by population cancer registries to derive internationally consistent and comparable information on childhood cancer stage at diagnosis using available medical records. The Guidelines are endorsed by the UICC TNM Prognostic Factors project and are published in the UICC TNM Classification of Malignant Tumours 8th Edition.(2) 4

6 General principles of the Toronto Paediatric Cancer Stage Guidelines 1. The Guidelines are intended for use by population registries only. The staging systems recommended in the Toronto Paediatric Cancer Stage Guidelines are intended for use by population cancer registries. They are not intended to replace staging systems in clinical use nor to conflict with the stage used by clinicians in determining the treatment and prognosis of individual patients. 2. Stage is a measure of extent of disease at diagnosis. The staging systems described are intended to be a measure the anatomic extent of disease at time of diagnosis. Stage is one of many prognostic indicators. Other non-anatomical prognostic indicators that are important for individual patient management and risk assessment (e.g. immuno-phenotype in leukaemia or MYCN amplification in neuroblastoma) may be collected by registries as resources permit, however, for most of the disease groups outlined here, these items do not form part of the recommended staging systems. 3. The goal is to derive the best estimate of stage. The criteria provided herein are intended to enable registries to derive the best estimate of stage at diagnosis using available data sources. There are limitations inherent in collecting the data items required for staging from medical records, and assumptions may be required. However, the criteria provided here will enable a reasonable and consistent measure of stage suitable for epidemiological analysis and stratified comparisons at a population level. 4. Resource-specific tiered staging systems are endorsed. The Guidelines endorse a two-tiered approach that provides less detailed criteria for registries with limited resources and data access (Tier 1) and more detailed staging criteria for well-resourced cancer registries (Tier 2). Tier 2 stage categories may be collapsed to Tier 1 categories to preserve comparability across registries. This manual is concerned solely with staging at the Tier 1 level 5

7 Table 1: The Toronto Paediatric Cancer Stage Guidelines (TIER 1) Diagnostic group/subgroup Tier 1 stage Diagnostic group/subgroup Tier 1 stage Acute lymphoblastic leukaemia CNS negative CNS positive Osteosarcoma Localized Acute myeloid leukaemia CNS negative CNS positive Ewings sarcoma Localized Hodgkin lymphoma Ann Arbor-stage IA/B Ann Arbor-stage IIA/B Ann Arbor-stage IIIA/B Ann Arbor-stage IVA/B Retinoblastoma Localized Regional Non-Hodgkin lymphoma Limited Advanced Hepatoblastoma Localized Neuroblastoma Localized Locoregional INRGSS-MS disease Testicular cancer Localized Regional Wilms tumour Localized Ovarian cancer Localized Regional Rhabdomyosarcoma Localized Medulloblastoma and other CNS embryonal tumours Localised Nonrhabdomyosarcoma soft-tissue sarcoma Localized Ependymoma Localized 6

8 General rules of staging 1. Stage is defined as extent of disease at the time of diagnosis and is based on evidence acquired before treatment (with the exception of Wilms tumour, see page xx). 2. For all diagnostic groups including Wilms tumour, the presence of distant metastases is assessed clinically or pathologically at the time of diagnosis and before neoadjuvant therapy. 3. If the medical record is incomplete in respect to the data items required for staging, stage is assessed as being unknown. 4. If the medical record is complete and there is no mention of a data item in the record, then it should be assumed that the item is negative/absent; for example: - if there is no mention in the medical record of metastases then assume no metastases ; - if there is no mention in the medical record of nodal involvement, then assume no nodal involvement. 5. If stage at the moment of diagnosis (before treatment by chemotherapy or radiotherapy) has already been explicitly recorded as TNM codes, the Paediatric Stage Equivalent can be determined from Table 2 Table 2: Paediatric Cancer Stage: TNM Equivalents 6. Codes for Wilms tumour Stage Code TNM Localised L Any T, any N, M0 M Any T, any N, M1 7,8 Codes for Soft tissue sarcomas Stage Code TNM Localised L Any T, any N, M0 M Any T, any N, M1 9,10 Codes for Osteosarcoma & Ewings sarcoma Stage Code TNM Localised L Any T, any N, M0(-) M Any T, any N, M1(+) [ for C40.2 : T4, any N, Mo] 11 Codes for Retinoblastoma Stage Code TNM Localised L T1-3, N0, M0 Regional R T4, N0, M0 Any T, N1, M0 M Any T, any N, M1 7

9 12 Codes for Liver tumours Stage Code TNM Localised L T1-3, any N, M0(-) M T4, any N, M0 Any T, any N, M1 13 Codes for Testicular cancers Stage Code TNM Localised L Any T, N0, M0 Regional R Any T, N1, M0 M Any T, any N, M1 14 Codes for Ovarian cancers Stage Code TNM Localised L T1, N0, M0 Regional R T2-3, N0, M0 Any T, N1, M0 M Any T, any N, M1 8

10 1. Acute lymphoblastic leukaemia International Classification of Childhood Cancer Third Edition (3) 1a Lymphoid leukaemias: 9820, (9823), 9826, 9827, , 9940, 9948 ICD-O-3 Sites: C000-C809 Morphology codes: Lymphoid leukaemia, NOS 9820/3 Burkitt cell leukaemia 9826/3 Adult T-cell leukaemia/lymphoma (HTLV-1 positive) 9827/3 T cell large granular lymphocytic leukaemia 9831/3 Prolymphocytic leukaemia, NOS 9832/3 Prolymphocytic leukaemia, B-cell type 9833/3 Prolymphocytic leukaemia, T-cell type 9834/3 Precursor cell lymphoblastic leukaemia, NOS 9835/3 Precursor B-cell lymphoblastic leukaemia 9836/3 Precursor T-cell lymphoblastic leukaemia 9837/3 Hairy cell leukaemia 9940/3 Agressive NK-cell leukaemia 9948/3 Acute lymphoblastic leukaemia Tier 1 is based on the extent of central nervous system (CNS) involvement. Definitions and notes CSF reports - If blasts are referred to as occasional or seen or similar wording, assume blasts are present. - If there is no mention of blasts, assume blasts are absent. Clinical signs of CNS involvement are defined as - Radiologic evidence of intracranial, intradural mass - Cranial nerve palsy (e.g. facial weakness, ptosis), brain/eye involvement or hypothalamic syndrome. Extra-ocular orbital masses, severe headaches and eye swelling (in the absence of signs of cranial nerve involvement) are not sufficient to constitute CNS involvement. Staging rules for acute lymphoblastic leukaemia CNS- No clinical signs of CNS involvement and no blasts in CSF CNS+ Clinical signs of CNS involvement or blasts in CSF Database entry codes for acute lymphoblastic leukaemia Stage Code CNS - C- CNS+ C+ 9

11 2. Acute myeloid leukaemia International Classification of Childhood Cancer Third Edition 1b - Acute myeloid leukaemias: 9840, 9861, , , 9891, , , 9920, 9931 ICD-O-3 Sites: C000-C809 Morphology codes: Acute myeloid leukaemia, M6 type 9840/3 Acute myeloid leukaemia, NOS 9861/3 Acute myeloid leukaemia with t(6;9)(p23;q34); DEK-NUP /3 Acute promyelocytic leukaemia, t(15;17)(q22;q11-12) 9866/3 Acute myelomonocytic leukaemia, NOS 9867/3 Acute myeloid leukaemia with inv(3)(q21q26.2) or t(3;3)(q21;q26.2); RPN1-EVII 9869/3 Acute basophilic leukaemia 9870/3 Acute myeloid leukaemia with abnormal marrow eosinophils 9871/3 Acute myeloid leukaemia, minimal differentiation 9872/3 Acute myeloid leukaemia without maturation 9873/3 Acute myeloid leukaemia with maturation 9874/3 Acute monocytic leukaemia 9891/3 Acute myeloid leukaemia with myelodysplasia-related changes 9895/3 Acute myeloid leukaemia, t(8;21)(q22;q22) 9896/3 Acute myeloid leukaemia, 11q23 abnormalities 9897/3 Myeloid leukaemia associated with Down syndrome 9898/3 Acute megakaryoblastic leukaemia 9910/3 Acute myeloid leukaemia (megakaryoblastic) with t(1;22)(p13;q13); RBM15-MKL1 9911/3 Therapy-related myeloid neoplasm 9920/3 Acute panmyelosis with myelofibrosis 9931/3 Acute myeloid leukaemia Tier 1 staging is based on whether there is central nervous system (CNS) involvement. Definitions and notes Traumatic and non-traumatic lumbar puncture - If RBC in CSF < 10/µL then lumbar puncture is nontraumatic. - If RBC in CSF 10/µL then lumbar puncture is traumatic. CSF reports - If blasts are referred to as occasional or seen or similar wording, assume blasts are present. - If there is no mention of blasts, assume blasts are absent. Clinical signs of CNS involvement are defined as - Radiologic evidence of intracranial, intradural mass - Cranial nerve palsy (e.g. facial weakness, ptosis), brain/eye involvement or hypothalamic syndrome. Extra-ocular orbital masses, severe headaches and eye swelling (in the absence of signs of cranial nerve involvement) are not sufficient to constitute CNS involvement. 10

12 CNS- CNS+ Staging rules for acute myeloid leukaemia Lumbar puncture nontraumatic (see Definitions and notes) and no blasts in CSF and no clinical signs of CNS involvement Lumbar puncture traumatic or Lumbar puncture nontraumatic and blasts in CSF or Clinical signs of CNS involvement Database entry codes for acute myeloid leukaemia Stage Code CNS - C- CNS+ C+ 11

13 3. Hodgkin lymphoma International Classification of Childhood Cancer Third Edition 2a - Hodgkin Lymphoma: , 9659, , 9667 ICD-O-3 Sites: C000-C809 Morphology codes: Hodgkin lymphoma, NOS 9650/3 Hodgkin lymphoma, lymphocyte-rich 9651/3 Hodgkin lymphoma, mixed cellularity, NOS 9652/3 Hodgkin lymphoma, lymphocyte depletion, NOS 9653/3 Hodgkin lymphoma, lymphocyte depletion, diffuse fibrosis 9654/3 Hodgkin lymphoma, lymphocyte depletion, reticular 9655/3 Hodgkin lymphoma, nodular lymphocyte predominance 9659/3 Hodgkin granuloma 9661/3 Hodgkin sarcoma 9662/3 Hodgkin lymphoma, nodular sclerosis, NOS 9663/3 Hodgkin lymphoma, nodular sclerosis, cellular phase 9664/3 Hodgkin lymphoma, nodular sclerosis, grade /3 Hodgkin lymphoma, nodular sclerosis, grade /3 Hodgkin lymphoma Tier 1 is identical to the Ann Arbor staging system.(4) Definitions and notes Nodal regions, extra-lymphatic organs or sites Staging requires assessment of - the number of nodal regions involved, by anatomical location (i.e., above or below the diaphragm). Nodal regions are listed in Figures 1a and 1b. - the number of extra-lymphatic organs or sites involved, by anatomical location (i.e., above or below the diaphragm). Constitutional symptoms The suffix A or B is added to the stage according to the absence or presence of defined constitutional symptoms, as follows: A = no constitutional symptoms are recorded, or the medical record states there are no constitutional symptoms B = medical record states there are constitutional symptoms Constitutional symptoms are: Fevers. Unexplained fever with temperature above 38 degrees Celsius (100.4 degrees F). Night sweats. Drenching sweats (e.g. those that require change of bedclothes). Weight loss. Unexplained weight loss of more than 10% of usual body weight in the 6 months prior to diagnosis. 12

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16 Staging rules for Hodgkin lymphoma Note: The suffix A or B is added to the stage according to the absence or presence of defined constitutional symptoms, as follows: A = no constitutional symptoms are recorded, or the medical record states there are no constitutional symptoms B = medical record states there are constitutional symptoms Constitutional symptoms are: Fevers. Unexplained fever with temperature above 38 degrees Celsius (100.4 degrees F). Night sweats. Drenching sweats (e.g. those that require change of bedclothes). Weight loss. Unexplained weight loss of more than 10% of usual body weight in the 6 months prior to diagnosis. Stage I Stage II Stage III Stage IV TIER 1 Involvement of a single lymph node region or Involvement of a single extralymphatic organ or site, without lymph node involvement. Involvement of two or more lymph node regions on the SAME side (either above or below) of the diaphragm or Localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement (i.e. local extension from a lymph node area into a nearby organ), with or without involvement of other lymph node regions on the SAME side (either above or below) of the diaphragm. Involvement of lymph node regions on BOTH sides (above and below) of the diaphragm. This may be accompanied by: - extralymphatic extension in association with adjacent lymph node involvement (i.e. local extension from a lymph node area into a nearby organ) and/or - involvement of spleen. Diffuse or disseminated involvement of one or more extralymphatic organs with or without associated lymph node involvement or Isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s). or Any involvement of liver, bone marrow, lungs (except by direct extension from another site) or CSF. 15

17 Database entry codes for Hodgkin lymphoma Stage Code Stage IA 1A Stage IB 1B Stage IIA 2A Stage IIB 2B Stage IIIA 3A Stage IIIB 3B Stage IVA 4A Stage IVB 4B 16

18 4. Non-Hodgkin lymphoma International Classification of Childhood Cancer Third Edition 2b Non-Hodgkin lymphomas (except Burkitt lymphoma): 9591, 9670, 9671, 9673, 9675, , 9684, , 9695, , 9705, 9708, 9709, 9714, , 9724, , , , , , c Burkitt lymphoma: 9687 ICD-O-3 Sites: C000-C809 (unless otherwise specified and excluding cutaneous lymphomas, C44_) Morphology codes: Ill-defined lymphomas and some mature B-cell lymphomas Malignant lymphoma, non-hodgkin, NOS 9591/3 Mature B-cell lymphomas Malignant lymphoma, small B lymphocytic, NOS 9670/3 Malignant lymphoma, lymphoplasmacytic 9671/3 Mantle cell lymphoma 9673/3 Malignant lymphoma, mixed small and large cell, diffuse 9675/3 Primary effusion lymphoma 9678/3 Mediastinal large B-cell lymphoma (C38.3) 9679/3 Malignant lymphoma, large B-cell, diffuse, NOS 9680/3 Malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS 9684/3 Burkitt lymphoma, NOS 9687/3 T-cell/histiocyte rich large B-cell lymphoma (was 9680/3 with rich ) 9688/3 Splenic marginal zone B-cell lymphoma (C42.2) 9689/3 Follicular lymphoma, NOS 9690/3 Follicular lymphoma, grade /3 Follicular lymphoma, grade /3 Follicular lymphoma, grade /3 Marginal zone B-cell lymphoma, NOS 9699/3 Mature T- and NK-cell lymphomas and some precursor cell neoplasms Mature T-cell lymphoma, NOS 9702/3 Angioimmunoblastic T-cell lymphoma 9705/3 Anaplastic large cell lymphoma, T-cell and Null cell type 9714/3 Hepatosplenic T-cell lymphoma 9716/3 Intestinal T-cell lymphoma 9717/3 Primary cutaneous CD30+ T-cell lymphoprolifera tive disorder 9718/3 NK/T-cell lymphoma, nasal and nasal-type 9719/3 Systemic EBV positive T-cell lymphoproliferative disease of childhood 9724/3 Precursor cell lymphoblastic lymphoma, NOS 9727/3 Precursor B-cell lymphoblastic lymphoma 9728/3 Precursor T-cell lymphoblastic lymphoma 9729/3 Plasma cell neoplasms and some mature B-cell lymphomas Plasmacytoma, NOS 9731/3 Multiple myeloma (C42.1) 9732/3 Plasma cell leukaemia (C42.1) 9733/3 Plasmacytoma, extramedullary 9734/3 Plasmablastic lymphoma (was 9684/3) 9735/3 ALK positive large B-cell lymphoma 9737/3 Large B-cell lymphoma arising in HHV8-associated multicentric 9738/3 17

19 Immunoproliferative neoplasms Immunoproliferative disease, NOS 9760/3 Waldenstrom macroglobulinaemia 9761/3 Heavy chain disease, NOS 9762/3 Immunoproliferative small intestinal disease (C17.-) 9764/3 Lymphoid leukaemias B-cell chronic lymphocytic lymphoma 9823/3 Adult T-cell leukaemia/lymphoma (HTLV-1 positive) 9827/3 Definitions and notes Staging requires assessment of CNS or bone marrow involvement Limited Advanced Staging rules for non-hodgkin lymphoma No involvement of CNS or bone marrow Involvement of CNS and/or bone marrow Database entry codes for non-hodgkin lymphoma Stage Code Limited L Advanced A 18

20 5. Neuroblastoma International Classification of Childhood Cancer Third Edition 4a Neuroblastoma and Ganglioneuroblastoma; 9490, 9500 ICD-O-3 Sites: C000-C809 Morphology codes: Ganglioneuroblastoma 9490/3 Neuroblastoma, NOS 9500/3 Tier 1 criteria do not require cross-sectional imaging Neuroblastoma Localized Locoregional Staging rules for neuroblastoma Localized tumour not involving vital structures and confined to one body compartment Locoregional tumour with spread Distant metastatic disease (ie, not contiguous with the primary tumour) except as defined for stage MS. Nonregional (distant) lymph node involvement is metastatic disease. However, an upper abdominal tumour with enlarged lower mediastinal nodes or a pelvic tumour with inguinal lymph node involvement is considered locoregional disease. Ascites and/or a pleural effusion, even with malignant cells, do not constitute metastatic disease unless they are remote from the body compartment of the primary tumour. MS disease confined to skin, liver, and/or bone marrow in a patient less than 18 months (547 days). Stage Limited Loco regional MS Database codes for Neuroblastomes Code L LR M MS 19

21 6. Wilms tumour International Classification of Childhood Cancer Third Edition 6a Nephroblastoma; 8959, 8960 ICD-O-3 Sites: C649 Morphology codes: Malignant cystic nephroma (C64.9) 8959/3 Nephroblastoma, NOS (C64.9) 8960/3 Definitions and notes In cases of bilateral disease - the presence of synchronous disease should be noted (LATERALITY = 2) - for purpose of staging, only the most advanced kidney should be recorded. At diagnosis, if diagnostic imaging reports on the status of the liver, lung, bone, brain and other sites and mention the words suspicious, highly suspicious, possible or highly suspected, record as metastatic disease (M) regardless of upfront surgery or chemotherapy. Staging rules for Wilms tumour Localized confined to area of origin Tumour Distant metastases present at diagnosis Database entry codes for Wilms tumour Stage Localised Code L M 20

22 7. Rhabdomyosarcoma International Classification of Childhood Cancer Third Edition 9a Rhabdomyosarcomas: , 8910, 8912, 8920, 8991 ICD-O-3 Sites: C000-C809 Morphology codes: Myomatous neoplasms Rhabdomyosarcoma, NOS 8900/3 Pleomorphic rhabdomyosarcoma, adult 8901/3 Mixed type rhabdomyosarcoma 8902/3 Fetal rhabdomyoma (coded as benign in Australia) 8903/x Adult rhabdomymoa (coded as benign in Australia) 8904/x Genital rhabdomyoma (coded as benign in Australia) 8905/x Embryonal rhabdomyosarcoma, NOS 8910/3 Spindle cell rhabdomyosarcoma 8912/3 Alveolar rhabdomyosarcoma 8920/3 Complex mixed and stromal neoplasms Embryonal sarcoma 8991/3 Localized Staging rules for rhabdomyosarcoma Tumour confined to the area of origin including the regional lymph nodes. Distant metastases present Database entry codes for rhabdomyosarcoma Stage Localised Code L M 21

23 8. Non-rhabdomyosarcoma soft-tissue sarcoma International Classification of Childhood Cancer Third Edition 9b Fibrosarcomas, peripheral nerve sheath tumours, and other fibrous neoplasms: 8810, 8811, , 8821, 8823, , 8822, , 9150, 9160, 9491, , d Other specified soft tissue sarcomas: 8587, , 8806, , 8836, , , , 8870, 8880, 8881, , 8921, 8963, 8982, 8990, , , , 9135, 9136, 9141, 9142, 9161, , 9210, 9220, 9231, 9240, 9251, 9252, 9260, 9364, 9365, 9373, e- Unspecified soft tissue sarcomas * Morphology 9180/3 is not included with 9d, but is in Osteosarcoma group ICD-O-3 Sites: C000-C809 (unless otherwise specified) Morphology codes: Thymic epithelial neoplasm Ectopic hamartomatous thymoma (coded as benign in Australia) 8587/x Paragangliomas and glomus Glomangiosarcoma 8710/3 Glomus tumour, malignant 8711/3 Glomangioma 8712/x Glomangiomyoma (coded as benign in Australia) 8713/x Soft tissue tumours and sarcomas, NOS Sarcoma, NOS (Sites: C000-C399, C440-C768, C809) 8800/3 Spindle cell sarcoma (Sites: C000-C399, C440-C768, C809) 8801/3 Giant cell sarcoma (Sites: C000-C399, C440-C768, C809) 8802/3 Small cell sarcoma (Sites: C000-C399, C440-C768, C809) 8803/3 Epithelioid sarcoma (Sites: C000-C399, C440-C768, C809) 8804/3 Undifferentiated sarcoma (Sites: C000-C399, C440-C768, C809) 8805/3 Desmoplastic small round cell tumour 8806/3 Fibromatous neoplasms Fibrosarcoma, NOS (Sites: C000-C399, C440-C768, C809) 8810/3 Fibromyxosarcoma (Sites: C000-C399, C440-C768, C809) 8811/3 Fascial fibrosarcoma (Sites: C000-C399, C440-C768, C809) 8813/3 Infantile fibrosarcoma (Sites: C000-C399, C440-C768, C809) 8814/3 Solitary fibrous tumour, malignant (Sites: C000-C399, C440-C768, C809) 8815/3 Elastofibroma 8820/x Aggressive fibromatosis (Sites: C000-C399, C440-C768, C809) 8821/x Abdominal fibromatosis 8822/x Desmoplastic fibroma (Sites: C000-C399, C440-C768, C809) 8823/x Myofibromatosis 8824/x Myofibroblastic tumour, NOS 8825/x Angiomyofibroblastoma 8826/x Myofibroblastic tumour, peribronchial 8827/x Malignant fibrous histiocytoma (Sites: C000-C399, C440-C768, C809) 8830/3 Histiocytoma, NOS 8831/x Dermatofibrosarcoma, NOS (C44._) 8832/3 Pigmented dermatofibrosarcoma protuberans (C44._) 8833/3 Giant cell fibroblastoma (Sites: C000-C399, C440-C768, C809) 8834/x Plexiform fibrohistiocytic tumour (Sites: C000-C399, C440-C768, C809) 8835/x 22

24 Angiomatoid fibrous histiocytoma 8836/x Myxomatous neoplasms Myxosarcoma 8840/3 Angiomyxoma, NOS 8841/x Ossifying fibromyxoid tumour 8842/x Lipomatous neoplasms Liposarcoma, NOS 8850/3 Well differentiated liposarcoma, NOS 8851/3 Myxoid liposarcoma 8852/3 Round cell liposarcoma 8853/3 Pleomorphic liposarcoma 8854/3 Mixed liposarcoma 8855/3 Fibroblastic liposarcoma 8857/3 Dedifferentiated liposarcoma 8858/3 Angiomyolipoma 8860/x Angiolipoma, NOS 8861/x Chondroid lipoma 8862/x Myelolipoma 8870/x Hibernoma 8880/x Lipoblastomatosis 8881/x Myomatous neoplasms Leiomyosarcoma, NOS 8890/3 Epithelioid leiomyosarcoma 8891/3 Cellular leiomyoma 8892/x Bizarre leiomyoma 8893/x Angiomyosarcoma 8894/3 Myosarcoma 8895/3 Myxoid leiomyosarcoma 8896/3 Metastasising leiomyoma 8898/x Rhabdomyosarcoma with ganglionic differentiation 8921/3 Complex mixed and stromal neoplasms Malignant rhabdoid tumour (Sites: C000-C639, C659-C699, C739-C768, C809) 8963/3 Malignant myoepithelioma 8982/3 Mesenchymoma, malignant 8990/3 Synovial-like neoplasms Synovial sarcoma, NOS 9040/3 Synovial sarcoma, spindle cell 9041/3 Synovial sarcoma, epithelioid cell 9042/3 Synovial sarcoma, biphasic 9043/3 Clear cell sarcoma, NOS 9044/3 Blood vessel tumours Haemangiosarcoma 9120/3 Cavernous haemangioma 9121/x Venous haemangioma 9122/x Racemose haemangioma 9123/x Kupffer cell sarcoma 9124/3 Epithelioid haemangioma 9125/x Haemangioendothelioma, malignant 9130/3 23

25 Capillary haemangioma 9131/x Intramuscular haemangioma 9132/x Epithelioid haemangioendothelioma, malignant 9133/3 Endovascular papillary 9135/x Spindle cell haemangioendothelioma 9136/x Angiokeratoma 9141/x Verrucous keratotic haemangioma 9142/x Haemangiopericytoma, malignant 9150/3 Angiofibroma, NOS 9160/x Acquired tufted haemangioma 9161/x Haemangioblastoma 9161/x Lymphatic vessel tumours Lymphangiosarcoma 9170/3 Capillary lymphangioma 9171/x Cavernous lymphangioma 9172/x Cystic lymphangioma 9173/x Lymphangiomyoma 9174/x Lymphangiomyomatosis 9174/x Haemolymphangioma 9175/x Osseous and chondromatous neoplasms Osteochondroma (Sites: C490-C499) 9210/x Osteochondromatosis, NOS (Sites: C490-C499) 9210/x Chondrosarcoma, NOS (Sites: C490-C499) 9220/3 Myxoid chondrosarcoma 9231/3 Mesenchymal chondrosarcoma (Sites: C490-C499) 9240/3 Giant cell tumours Malignant giant cell tumour of soft 9251/3 Malignant tenosynovial giant cell tumour 9252/3 Miscellaneous tumours Parachordoma 9373/x Neuroepitheliomatous neoplasms Ganglioneuromatosis 9491/x Nerve sheath tumours Neurofibromatosis, NOS 9540/x Malignant peripheral nerve sheath tumour 9540/3 Neurilemoma, malignant 9560/3 Malignant peripheral nerve sheath tumour with rhabdomyoblastic differentiation 9561/3 Perineurioma, malignant (MPNST) 9571/3 Granular cell tumours and alveolar soft part sarcomas Granular cell tumour, malignant 9580/3 Alveolar soft part sarcoma 9581/3 24

26 Localized Staging rules for non- rhabdomyosarcoma soft tissue sarcomas Tumour confined to the area of origin including the regional lymph nodes. Distant metastases present Database entry codes for non-rhabdomyosarcoma soft tissue sarcomas Stage Code Localised L M 25

27 9. Osteosarcoma International Classification of Childhood Cancer Third Edition 8a Osteosarcoma: , , 9200 ICD-O-3 Sites: C400-C419, C760-C768, C809 Morphology codes: Osteosarcoma, NOS 9180/3 Chondroblastic osteosarcoma 9181/3 Fibroblastic osteosarcoma 9182/3 Telangiectatic osteosarcoma 9183/3 Osteosarcoma in Paget disease of bone 9184/3 Small cell osteosarcoma 9185/3 Central osteosarcoma 9186/3 Intraosseous well differentiated osteosarcoma 9187/3 Osteoid osteoma, NOS 9191/x Parosteal osteosarcoma 9192/3 Periosteal osteosarcoma 9193/3 High grade surface osteosarcoma 9194/3 Intracortical osteosarcoma 9195/3 Osteoblastoma, NOS 9200/x Definitions and notes Skip lesions, skip metastases or seeding in the same bone as the primary tumour are considered localized and not metastatic; if in a different bone to the primary tumour these are considered metastatic. Localized Staging rules for osteosarcoma Tumour confined to the area of origin including regional lymph nodes Distant metastases present Database entry codes for Osteosarcomas Stage Localised Inconnu Code L M 26

28 10. Ewing sarcoma International Classification of Childhood Cancer Third Edition 8c Ewing tumour and related sarcomas of bone: 9260, ICD-O-3 Sites: C400-C419, C760-C768, C809 Morphology: Ewing sarcoma (Sites: C000-C809) 9260/3 Melanotic neuroectodermal tumour 9363/x Peripheral neuroectodermal tumour, NOS 9364/3 Askin tumour 9365/3 Localized Staging rules for Ewing sarcoma Tumour confined to the area of origin including regional lymph nodes Distant metastases present Stage Localised Database entry codes for Ewing sarcoma Code L M 27

29 11. Retinoblastoma International Classification of Childhood Cancer Third Edition 5 Retinoblastoma: ICD-O-3 Sites: C692 Morphology codes: Retinoblastoma, NOS 9510/3 Retinoblastoma, differentiated 9511/3 Retinoblastoma, undifferentiated 9512/3 Retinoblastoma, diffuse 9513/3 Retinoblastoma, spontaneously regressed (coded as uncertain in Australia) 9514/x Definitions and notes In cases of bilateral disease: - the presence of synchronous disease should be noted (LATERALITY = 2) - for purpose of stage, only the most advanced eye should be recorded. Localized Regional Staging rules for retinoblastoma Intraocular Orbital extension or regional lymph nodes Distant metastases present Database entry codes for retinoblastoma Stage Localised Regional Code L R M 28

30 12. Hepatoblastoma International Classification of Childhood Cancer Third Edition 7a Hepatoblastoma: 8970 ICD-O-3 Site: C220 Morphology: Hepatoblastoma, NOS 8970/3 Localized Staging rules for hepatoblastoma Tumour confined to the area of origin including regional lymph nodes Distant metastases present Database entry codes for hepatoblastoma Stage Localised Code L M 29

31 13. Testicular cancer International Classification of Childhood Cancer Third Edition 10c Malignant gonadal germ cell tumours: , , , , 9100, 9101 ICD-O-3 Sites: C620-C629 Morphology codes: Germ cell neoplasms Dysgerminoma 9060/3 Seminoma, NOS 9061/3 Seminoma, anaplastic 9062/3 Spermatocytic seminoma 9063/3 Germinoma 9064/3 Germ cell tumour, nonseminomatous 9065/3 Embryonal carcinoma, NOS 9070/3 Yolk sac tumour, NOS 9071/3 Polyembryoma 9072/3 Gonadoblastoma 9073/x Teratoma, malignant, NOS 9080/3 Teratocarcinoma 9081/3 Malignant teratoma, undifferentiated 9082/3 Malignant teratoma, intermediate 9083/3 Teratoma with malignant transformation 9084/3 Mixed germ cell tumour 9085/3 Struma ovarii, malignant 9090/3 Strumal carcinoid 9091/x Trophoblastic neoplasms Choriocarcinoma, NOS 9100/3 Choriocarcinoma combined with other germ cell elements 9101/3 Localized Regional Tumour confined to the testes Staging rules for testicular cancer Tumour extension to regional lymph nodes: - Interaortocaval - Para-aortic (periaortic) - Paracaval - Preaortic - Precaval - Retroaortic - Retrocaval - Along spermatic cord Distant metastases present 30

32 Database entry codes for testicular cancer Stage Localised Regional Code L R M 31

33 14. Ovarian cancer International Classification of Childhood Cancer Third Edition 10c Malignant gonadal germ cell tumours: , , , , 9100, 9101 ICD-O-3 Site: C569 Morphology codes: Germ cell neoplasms Dysgerminoma 9060/3 Seminoma, NOS 9061/3 Seminoma, anaplastic 9062/3 Spermatocytic seminoma 9063/3 Germinoma 9064/3 Germ cell tumour, nonseminomatous 9065/3 Embryonal carcinoma, NOS 9070/3 Yolk sac tumour, NOS 9071/3 Polyembryoma 9072/3 Gonadoblastoma 9073/x Teratoma, malignant, NOS 9080/3 Teratocarcinoma 9081/3 Malignant teratoma, undifferentiated 9082/3 Malignant teratoma, intermediate 9083/3 Teratoma with malignant transformation 9084/3 Mixed germ cell tumour 9085/3 Struma ovarii, malignant 9090/3 Strumal carcinoid 9091/x Trophoblastic neoplasms Choriocarcinoma, NOS 9100/3 Choriocarcinoma combined with other germ cell elements 9101/3 Localized Regional Staging rules for ovarian cancer Tumour confined to ovaries Tumour involves one or both ovaries with pelvic extension and/or spread to the peritoneum outside the pelvis and/or retroperitoneal lymph nodes Distant metastatic disease excluding peritoneal metastases Stage Localised Regional Database entry codes for ovarian cancer Code L R M 32

34 15. Medulloblastoma and other CNS embryonal tumours International Classification of Childhood Cancer Third Edition 3c. Intracranial and intraspinal embryonal tumours 9362, , 9480, , 9508 ICD-O-3 Sites: C700-C729, C753 Morphology codes: Miscellaneous tumour Pineoblastoma 9362/3 Medulloblastoma Medulloblastoma, NOS 9470/3 Desmoplastic nodular medulloblastoma 9471/3 Medullomyoblastoma 9472/3 Large cell medulloblastoma 9474/3 Cerebellar sarcoma, NOS 9480/3 Other Embryonal CNS tumours Primitive neuroectodermal tumour, NOS 9473/3 Medulloepithelioma Medulloepithelioma, benign (sites C700-C729) 9501/0 Medulloepithelioma, NOS (sites C700-C729) 9501/3 Teratoid medulloepithelioma, benign (sites C700-C729) 9502/0 Teratoid medulloepithelioma, NOS (sites C700-C729) 9502/3 Neuroepithelioma, NOS (sites C700-C729) 9503/3 Spongioneuroblastoma (sites C700-C729) 9504/3 Atypical teratoid/rhabdoid tumours Atypical teratoid/rhabdoid tumour 9508/3 Localized Staging rules for medulloblastoma Localized disease Disease beyond local site (e.g., other lesions in brain or spine, tumour cells in CSF or distant metastases Database entry codes for Medulloblastomes Stage Localised Code L M 33

35 16. Ependymoma International Classification of Childhood Cancer Third Edition 3a Ependymomas: 9383, ICD-O-3 Sites: C710-C729 Morphology codes: Subependymoma 9383/1 Ependymoma, NOS 9391/3 Ependymoma, anaplastic 9392/3 Papillary ependymoma 9393/3 Myxopapillary ependymoma 9394/1 Localized Localized disease Staging rules for Ependymoma Disease beyond local site (e.g., other lesions in brain or spine, tumour cells in CSF or distant metastases Stage Localised Database entry codes for Ependymomas Code L M 34

36 References 1. Gupta S, Aitken JF, Bartels U, Brierley J, Dolendo M, Friedrich P, et al. Paediatric cancer stage in populationbased cancer registries: the Toronto consensus principles and guidelines. The Lancet Oncology. 2016;17(4):e Brierley J, Gospodarowicz M, Wittekind C, editors. The TNM Classification of Malignant Tumours, 8th edition. Lyon, France: Union for International Cancer Control (UICC); Steliarova-Foucher E, Stiller C, Lacour B, Kaatsch P. International Classification of Childhood Cancer, third edition. Cancer. 2005;103(7): Carbone PP, Kaplan HS, Musshoff K, Smithers DW, Tubiana M. Report of the Committee on Hodgkin's Disease Staging Classification. Cancer Res. 1971;31(11):

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