Int. J. Cancer:68,759-765 (1996) 8 1996 Wiley-Liss, Inc. ‘ @ && *-- Publication of the International Unwn Against Cancer Publication de I’Union Internalionale Contre le Cancer THE INTERNATIONAL CLASSIFICATION OF CHILDHOOD CANCER E. KRAMAROVA’,~ and C.A. STILLER~ I Cancer Research Institute, Slovak Academy of Sciences, Bratislava, Slovakia; and ‘Childhood Cancer Research Group, University of oxford, Depallment of Paediatncs, 57 Woodstock Road, Oxford OX2 6HJ, UK. The International Classification of Childhood Cancer (ICCC) updates the widely used Birch and Marsden classification scheme. ICCC is based on the second edition of the International Classification of Diseases for Oncology (ICD-0-2). The purpose of the new classification is to accommodate important changes in recognition of different types of neoplasms, while preservingcontinuity with the original classification. The grouping of neoplasms into I 2 main diagnostic groups is maintained. The major changes are: (I) intracranial and intraspinal germcell tumours now constitute a separate subgroup within germcell tumours; (2) histiocytosis X (Langerhans-cell histiocytosis) is excluded from ICCC; (3) Kaposi’s sarcoma is a separate subgroup within soft-tissue sarcomas; (4) skin carcinoma is a separate subgroup within epithelial neoplasms; (5) “other specified” and “unspecified” neoplasms are now usually separate subcategories within the main diagnostic groups. Draft copies of the ICCC were distributed to some 200 professionals with interest and expertise in the field and their comments are consideredin this final version. This classification will be used for presentation of data in the second volume of the IARC Scientific Publication “Internotional Incidence of Childhood Cancer.’’ A computer progmmme for automated classification of childhood tumours coded according to ICD-0-1 or ICD-0-2 is now available from IARC. Q I996 Wiley-Liss,Inc. In the International Classification of Diseases (ICD), cancers other than leukacmias, lymphomas and melanomas arc categorized solely by site of origin. Among adults, the great majority of cancers are carcinomas (Miller and Myers, 1983) and so the ICD is satisfactory for presenting data on incidence, survival and mortality. Cancers in childrcn, however, arc histologically very divcrse and some histological types of childhood tumour occur in many different sites. It is firmly established that, in consequence, the classification of childhood cancer should be based on morphology. Several different classification systems havc been used in the past, but that of Birch and Marsden (1987), was used for the presentation o f the data in the IARC study “International Incidence of Childhood Cancer,” Vol. 1 (IICC-1) (Parkin et al., 1988) and has subsequently bcen widely accepted as a standard. The introduction of the 2nd edition of the International Classification of Diseases for Oncology (ICD-0-2) (Percy et al., 1990) and the 10th revision of the ICD (1992) necessitated an updating of the Birch and Marsden classification, to allow for the new and expanded coding of cancer which these havc introduced. The opportunity was therefore taken to introduce a few modifications which were dccmed to be essential, while keeping the new scheme for childhood cancer as close as possible to the original. A detailed account of the classification is being published as an IARC Technical Report (Kramarova et al., 1996). The aims of the present paper arc to introduce the new scheme, to document the main ways in which it differs from the original Birch and Marsden schcme and to relate thcse modifications to recent developments in the pathology and epidemiology of childhood cancer. To ensurc that thc revised scheme meets as many as possible of the needs of users, draft versions were widely circulated to professionals working in paediatric oncology and to those concerned with cancer epidemiology and cancer registration (espccially for the childhood age-range). All comments werc considered by the editorial group. This revised International Classification of Childhood Cancer therefore appears under the joint auspiccs of International Agency for Rescarch on Cancer (IARC), the International Association of Cancer Registries (IACR) and the International Society of Paediatric Oncology (SIOP). THE REVISED CLASSIFICATION The International Classification of Childhood Canccr (ICCC) is based on the ICD-0-2 morphology and topography codes. The division into 12 major diagnostic groups remains as in the original Birch-Marsdcn scheme. The only transfer between these groups concerns nonmalignant intracranial and intraspinal germ-cell tumours, which were formerly included in Group 111 (CNS and miscellaneous intracranial and intraspinal neoplasms) and now appear in Group X (germ-cell, trophoblastic and other gonadal neoplasms). Within the main diagnostic groups, the categories of “other specified” and “unspecified” neoplasms are now usually distinguished. The former subgroup “histiocytosis X” has been withdrawn entirely. Many more histological types, including those new to the ICD-0-2, have bcen added to the subgroups of carcinomas, including those new to the ICD-0-2, as in theory almost any histological type can appear in any topographic site. The complete classification rules arc given in the Appendix. I. Leukaemias All lymphoid leukaemias are grouped together. In practice, this group corresponds to acute lymphocytic lcukaemia (ALL), since chronic lymphocytic leukacmia virtually never occurs in children. Investigation of data sets submitted for IICC-1 had shown that, in thc former classification scheme, the category “other lymphoid leukaemia” (Ib) was either very small, or contained mainly cases of unspecified lymphoid leukaemia. Inclusion of a category for those cases, almost all of which would probably be ALL, had the cffect of introducing artificial differences in the incidence of acute lymphocytic leukaemia (formerly Ia), and therefore the distinction between the 2 groups has been eliminated. Thc group of acute non-lymphocyticleukaemia corrcsponds to the diagnoses included in thc FAB classification of acute myeloid lcukaemia (Bennett et al., 1985). It includes acute myelomonocytic leukaemia, which did not havc a separate code in ICD-0-1, and acute megakaryoblastic leukaemia, which was previously classified among other and unspecified Icukaemias. Chronic myeloid leukaemia (CML) includes both juvenile and adult CML, which share a common morphology code, together with chronic myelomonocytic leukaemia, which has its own code for the first time in ICD-0-2. 3Present address and address for correspondence and reprint requests: Unit of Descri live Epidemiology, International A ency for Research on Cancer. 156)CoursAlbert Thomas, 69372 Lyon tedex 08, France. Fax: 00334 7273 8575. Received: August 5, 1996. 760 KRAMAROVAAND STILLER Other specified leukaemias and unspecified leukaemias have been made into separate subgroups. Among children, the former group consists overwhelmingly of myeloid leukaemia not specified as acute or chronic. 11. Lymphomas and other reticuloendothelial neoplasms Several classification systems are in use for lymphomas: Working Formulation (1982), Kiel (Gerard-Marchant et al., 1974; Stansfeld et af., 1988), Lukes-Collins (1974); the Revised European-American Classification of Lymphoid Neoplasms (REAL) has been proposed (Harris et af., 1994). Application of these classifications was considered for ICCC, but it was felt that the benefits of incorporating some of their principles were outweighed by the loss of continuity with the previous, widely accepted childhood classification. Hence, the differences between the 2 childhood classifications reflect mainly the substantial changes made in the ICD-0-2 to the codes for lymphomas. Hodgkin’s disease is still recognized as a well-defined entity in the REAL classification (Harris et al., 1994), and constitutes an important subgroup in ICCC. The coding of non-Hodgkin lymphomas in ICD-0-1 was completely revised for ICD-0-2. The subgroup includes malignant lymphoma, non-Hodgkin, not otherwise specified (NOS), malignant lymphoma, diffuse, NOS, and malignant lymphomas, specified type, diffuse o r NOS, with the exception of Burkitt’s lymphoma. Also included are follicular or nodular malignant lymphomas, specified cutaneous and peripheral T-cell lymphomas and other specified non-Hodgkin lymphomas, as well as true histiocytic lymphoma and the somewhat obsolete diagnoses of lymphosarcoma and reticulosarcoma. Burkitt’s lymphoma constitutes a separate subgroup. It can be readily distinguished from other subtypes of NHL by its histological appearance and cytogenetic features, with breakages and translocations on chromosome 8 (Shiramizu et al., 1991). It is of great epidemiological importance because of the well-documented large-scale variations in incidence between regions of the world, and its proven aetiological link with Epstein-Barr virus (EBV). The subgroup of miscellaneous lymphoreticularneoplasms is a heterogeneous grouping of malignant histiocytosis, plasma-cell tumours, mast-cell tumours and malignant immunoproliferative diseases. Unspecified lymphoma is given a separate cat%Ory. As mentioned above, Langerhans cell histiocytosis (LCH, formerly known as histiocytosis X, Group IIe in the Birch and Marsden classification, M9722 in I C D - 0 and M77860, M77910, M77920 in SNOMED) is no longer included, although we realise that not all clinicians will agree with this. Although frequently treated by oncologists, this group of disorders is no longer regarded as being neoplastic (Writing Group of the Histiocyte Society, 1987), though its precise nature is still uncertain (Rivera-Luna et al., 1996). Moreover, ascertainment by registries is usually very incomplete (Stiller and Parkin, 1990), partly because of variations in decision as to whether cases of multisystem LCH should be coded to I C D - 0 M9722 or to SNOMED M77910. 111. Central nervous system and miscellaneous intracranial and intraspinal neoplasms Contrary to the general rule, some nonmalignant ncoplasms are also included in this group because it is difficult to establish a borderline between benign and malignant neoplasms in the case of ependymomas, some gliomas. and miscellaneous and unspecified intracranial and intraspinal neoplasms (Ziilch et al., 1979). Many cancer registries routinely include non-malignant tumours of these sites. Ependymoma includes also subependymal glioma and choroid plexus papilloma. The astrocyfoma group contains astrocytomas, gliomatosis cerebri and glioma of the optic nerve. The concept of primitive neuroectodermal tumours (PNET) (Hart and Earle, 1973), characterized by a primitive histological appearance and aggressive behaviour, has been introduced into the classification. In ICCC, this term is used both for medulloblastoma and for morphologically indistinguishable neoplasms located at other sites from the medulloblastomas but within the CNS, as recommended by the W H O Working Group (Kleihues et al., 1993). This diagnostic subgroup thus includes medulloblastomas arising in the cerebellum, and PNETs occurring in the cerebrum, spinal cord and pineal gland. The group of othergliomas contains those gliomas, including oligodendroglioma, which are not classified elsewhere. The group of other specified intracranial and intraspinal neoplasms includes certain adenomas and adenocarcinomas which characteristically occur in the pituitary, together with craniopharyngioma, pineal tumours, cerebellar sarcoma, ganglioglioma and meningiomas. Unspecified intracranial and intraspinal neoplasms contain unspecified neoplasms, whether benign or malignant, occurring in the meninges, spinal cord, cranial nerves and other parts of central nervous system, in pituitary and pineal glands and in the craniopharyngeal duct. Non-malignant intracranial and intraspinal germ-cell tumours have been withdrawn from among CNS neoplasms. Because of their origin, they are more appropriately classified with germ-cell neoplasms. IV.Sympathetic nervous system tumours The subgroup neuroblastoma and ganglioneuroblastoma includes tumours ranging from undifferentiated neuroblastoma to fully differentiated ganglioneuroblastoma, derived from primordial neural-crest cells. They arise in the sympathetic ganglia, adrenal medulla and other sites (Brodeur and Castleberry, 1993). Other sympathetic nervous system turnours include malignant paragangliomas, glomus tumours and all other neurocpitheliomatous neoplasms, except for those classified with neuroblastoma and retinoblastoma. V. Retinoblastoma As in the previous classification (Birch and Masden, 1987), retinoblastoma constitutes a separate group because of its exceptional clinical, epidemiological and genetic importance. The group combines all histological types of retinoblastoma. VI. Renal tumours Renal tumours in children feature a wide variety of morphologic types, some of them nonmalignant. The malignant neoplasms are divided into 3 subgroups. The first comprises Wifms’ tumour and its histologically distinct variants, clear-cell sarcoma and rhabdoid tumour of the kidney (Fernbach and Feinstein, 1995). Mesoblastic nephroma is not included as it is not malignant and is not generally recorded by cancer registries. Renal carcinoma includes renal-cell carcinoma and a number of other carcinomas occurring in the kidney. A separate subgroup is maintained for unspec$ed malignant renal turnours. As before, other rare neoplasms occurring in the kidney, notably sarcoma, are grouped with tumours of the same histological type in all other sites. VII. Hepatic tumours Hepatic tumours are very rare in childhood. The embryonal hepatoblastoma is distinguished from hepatic carcinoma. Virtu- 761 CHILDHOOD CANCER CLASSIFICATION ally all carcinomas occurring in the liver of children arc hepatocellular carcinomas, but provision is made for many other histological types, including certain adenocarcinomas, which typically occur in the intra- o r extra-hepatic bile ducts. Sarcomas and germ-cell tumours of liver are classified in Groups IX and X respectively. VIII. Malignant bone tumours The most common malignant bone tumour of childhood is osteosarcoma, deriving from primitive bone-forming mesenchyma. Different subtypes, including small-cell osteosarcoma, form this diagnostic subgroup. Chrondrosarcoma, distinguished by lack of production of osteoid substance (Link and Eilber, 1992) stands as a separate subgroup. Ewing’s sarcoma of bone or of unspecified topography and peripheral neuroectodermal tumour in bone are grouped together because of their possible common genetic origin. Identical translocations between chromosomes 11 and 22 have been observed in these 2 tumour types (Dehner, 1993). Other specified malignant bone tum0ur.s and unspecified malignant bone turnours form 2 separate subgroups. IX. Soft-tissue sarcomas The soft-tissue sarcomas comprise malignant solid tumours of soft tissues arising in different cells. The most common are rhabdomyosarcomas arising from mcsenchymal tissue, which with embryonal sarcoma constitute one subgroup. Fibrosarcoma, neurofibrosarcoma and other fibromatous neoplasms include malignant fibrous histiocytoma and dermatofibrosarcoma. The group also contains nerve-sheath tumours, among them malignant triton tumour which has its own morphology code in ICD-0-2-as recommended by Marsden (1988). Periosteal fibrosarcoma, however, is included in “Other specified malignant bone tumours.” A new subgroup has been crcatcd for Kaposi’s sarcoma, in recognition of the increasing importance of this tumour in some regions of the world (Wabinga et al., 1993). The group other specified sop-tissue sarcomas contains a variety of histological types, among them, liposarcoma, leiomyosarcoma and various malignant vascular tumours. Ewing’s sarcoma of soft tissues and its more differentiated form, peripheral neuroectodcrmal tumour of soft tissues, are also included. Ewing’s sarcoma of soft tissue was formerly grouped with rhabdomyosarcoma, as its origin was believed to be endothelial, but recent evidence points to a neural origin for this tumour (Crist and Kun, 1991). Melanotic neuroectodcrma1 tumour of extra-skeletal tissues is also included in this group because of its common origin in ncural crest (Enzinger et al., 1969). The last subgroup, unspecified soft-tissue sarcoma, comprises unspecified soft-tissue tumours and sarcomas occurring in all extra-skeletal sites. other sites are grouped as other and unspecified non-gonadal germ-cell tumours. Gonadal carcinomas include multiple histologies (9 new to ICD-0-2) appearing in gonads. Other and unspecified malignant gonadal tumours include malignant forms of specialized gonadal ncoplasms and Brenncr tumour as well as unspecified neoplasms restricted topographically to the gonads. XI. Carcinomas and other malignant epithelial neoplasms This group brings together most of the different types of neoplasm, originating in the epithelium, which are in general not typical in childhood. The most distinctive of these are assigned separate subgroups. The difficulty of distinguishing histologically between benign and malignant adrenocortical tumours is well recognized; in ICD-0-2 there is only one code for adrenocortical carcinoma, but in order to allow for more specific coding of histological subtypes, the subgroup adrenocortical carcinoma in ICCC comprises all matrix codes for adrenal cortical tumours listed in ICD-0-2, if their behaviour is codcd as malignant. Although most thyroid cancers in children are papillary or follicular carcinomas, many other histological types topographically limited to the thyroid were also included in this subgroup. Nasophatyngeal carcinoma is a relatively important childhood tumour, particularly in South-cast Asia and Northern Africa (Stiller, 1994), and is also characteristically associated with EBV. The group malignant melanoma comprises, in addition to malignant melanoma of skin, malignant forms of naevi, and malignant melanomas occurring at any other site, notably ocular tumours. Among the former subgroup “other carcinomas”, the most numerous tumours were those occurring in the skin, which exceeded, for example, the numbers of adrenocortical carcinoma (Parkin et a/., 1988). Furthermorc, some registries deliberately exclude non-melanoma skin cancers. Grouping them with other carcinomas would result in the rates of this potentially important cancer remaining hidden and the rates of other carcinomas being obscured by differential reporting of skin cancers in individual registries. Skin carcinoma is therefore distinguished as a separate subgroup. The subgroup other and unspecified carcinomas is restricted to tumours that are not already included within other subgroups by virtue of their specific primary site; it contains a wide variety of histological types. XII. Other and unspecified malignant neoplasms This group of the remaining neoplasms is now divided into “specified” and “unspecified”. The subgroup other specified malignant tumours contains rare tumours, which are not thought to be appropriate for inclusion elsewhere. They include, for example, adenosarcoma, mesodermal mixed tumour, pancreatoblastoma, pulmonary blastoma, carcinosarcoma, mesonephroma and mcsothelioma. X. Germ-cell, trophoblastic and other gonadal neoplasms This diagnostic group brings together the neoplasms arising in the gonads (derived from sex stromal or epithelial cells) or in their embryonal precursors, the germ cells. Germ-cell tumours are divided into 3 diagnostic subgroups according to their topographical location. Intracranial and intraspinal germ-cell tumours are those occurring in the central nervous system, pituitary gland, craniopharyngeal duct and pineal gland. As with other neoplasms in these locations, tumours of benign or uncertain bchaviour are also included. Malignant germ-cell tumours of the ovary or testis form the group ofgonadalgerrn-celltumours, while those occurring in all COMPUTER PROGRAMME To facilitate the introduction of the new classification into practice, a computer programme, “CHILD-CHECK”, has been written, which classifies childhood neoplasms. Both editions of I C D - 0 arc convertible into ICCC. The programme also runs several routines designed to check individual cancer records for internal consistency. The programme checks the validity of the unique identification number, the defined codes for dates, age and sex, and the acceptability of the morphological and topographic codes. The user is able to scarch for unlikely combinations of tumour type with sex or age, and for unusual 762 MAMAROVAAND STILLER topographic codes for specific morphologies. The erroneous or unlikely records are printed in different files according to the type of enquiry. In the “result” file, containing all accepted records, the ICCC codes are appended to the end of the original records. A detailed description of CHILD-CHECK is included in the IARC Technical Report (Kramirova et ul., 1996), together with a diskette containing the programme. DISCUSSION The existence of a standard classification is essential for comparability between different regions and periods (Muir, 1995). As with all classification schemes, however, periodic revision is needed to allow for changes in nomenclature or knowledge of disease histogenesis, but this should be considered in the light of the desirability of continuity to permit comparative studies over time. The Birch and Marsden (1987) classification was the first internationally accepted classification of childhood cancers. However, it is based on ICD-0-1 and with the increase in use of ICD-0-2 it became necessary also to update it. The ICCC is intended to be a natural continuation of the former childhood classification. Except for the application of ICD-0-2 codes (including the newly recognized entitics), its construction was guided by balancing 2 main principles. Firstly, the classification was to be improved by accommodating advances in studies of tumour biology, pathology and epidemiology. Secondly, continuity with the Birch and Marsden scheme was to be preserved to the maximum possible extent. The differences between the 2 schemes are therefore restricted to those for which there is strong supporting scientific evidence. Although the 2 systems are highly comparable, exact translation is not always possible. The distinction between “other” and “unspecified” neoplasms in several diagnostic groups (I, leukaemias; 111, CNS neoplasms; VIII, malignant bone tumours; IX, soft-tissue sarcoma; and XII, other and unspecified) in the ICCC rules out the direct comparison of these subgroups. The move of the non-malignant intracranial and intraspinal germ-cell tumours from group 111 (CNS neoplasms) to group X (germ-cell tumours), and changes made in groups VIII (malignant bone tumours), IX (soft-tissue sarcomas) and XI (epithelial tumours) have to be taken into account when comparing rates. On the other hand, in the diagnostic subgroups for which the only change has been inclusion or exclusion of particular histological types, incidence rates arc likely to be much the same with either classification. In certain childhood tumours, the discrimination between malignant and benign behaviour is particularly difficult, notably for adrenocortical and thyroid tumours, melanomas and teratomas. Usually, cancer registries do not register c a w diagnosed as non-malignant by a pathologist. Exclusion of such neoplasms from ICCC thus reflects its main purpose, a uniform presentation of comparable childhood cancer data. It is conceivable that many of the diagnostic subgroups are not completely homogeneous with respect to their origin, nature, clinical behaviour, best treatment or outcome. Attempts have been made to develop classification systems which would ideally address all these tumour characteristics (brain tumours: Rorke el al., 1985; rhabdomyosarcoma: Tsokos, 1994). ICCC did not aspire to subdivide the childhood tumours into completely homogeneous categories. Further fractionation of the diagnostic groups would make the categories sparse and less comparable. This classification should provide a useful tool for comparing common or otherwise important groups of childhood neoplasms between regions and periods. Epidemiological studies of cancer in adolescents have shown that the childhood classification also describes the patterns of incidence in the 15-19 age group better than does the system used for adults (Fritschi et al., 1995). With minor modifications, confined to further splitting of the category “other and unspecified carcinomas”, the ICCC would be readily applicable to this age-group. This finding is supported also by the distribution of diagnostic groups among adolescents in Nordic countries (Tulinius et af., 1992; Martos et af., 1993). ACKNOWLEDGEMENTS The International Classification of Childhood Cancer (ICCC) was sponsored by the International Agency for Research on Cancer, the International Association of Cancer Registries and the International Society of Paediatric Oncology (SIOP). We are very grateful to all the members of SIOP and other clinicians and epidemiologists who commented on drafts of ICCC and to the co-authors of ICCC who commented on earlier versions of this documcnt. The Childhood Cancer Research Group is supported by the UK Department of Health and the Scottish Home and Health Department. We are grateful to Anita Bayne for secretarial assistance. 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Histological ty in8 of tumours of the central nervous system. International SHIKAMIZU, B.. BARRIGA,F., NEEOUAYE, J., JAVKI,A,, DALLA- histological dsssification of tumours, 21, World Health Organisation. FAVERA, R., NERI,A,, GUTTIERE%, M., LEVINE, P. and MAGKAI'H, I., Geneva (1 979). APPENDIX - IN?'tKNATIONAL CLASSIPICAIION OF CHILDHOOD CANCER (ICCC) ICD-0-2 codes Diagnostic group ToDoarauhv .Uorpholom I. c) Chronic myeloid leukaemia d) Other specified leukaemias (e) Unspecified leukaemias 11. Lymphomas and reticuloendothelial neoplasms 9820-9827.9850 9840,9841,9861,9864,9866,9867,9891, 9894, 9910 9863.9868 9830; 9842,9860,9862,9870-9890,9892, 9893,9Yo0,9930-9941 9800-9804 9650-9667 9591-9595,9670-9686,96(x)-Y714,9723 9687 9720,9731-9764 9590 111. CNS and miscellaneous intracranial and intrar]naI neoplasms a Ependymoma b Astrocytoma tumours (e) Other specified intracranial and intraspinal neoplasms ( f ) Unspecified intracranial and intraspinal neoplasms IV. Sympathetic nervous system tumours (a Neuroblastoma and ganglioneuroblastoma (b] Other sympathetic nervous system tumours V. Retinoblastoma VI. Renal tumours (a) Wilms' tumour, rhabdoid and clear-cell sarcoma (b) Renal carcinoma - f c ) UnsDecified malignant renal tumours \ , 9383,9390-9394' 9380 9381,940&9441 9470-9473 9380 9382,9384l 9442-9460.9481 8270-8281; 8300,9350-9362,9480,9505, 9530-95392 8000-80042 C72.3 C70.0472.2, C72.4C72.9 C70.M72.9, C75.1LC75.3 9490,9500 8680,8693-8710,9501-9504,9520-9523 95 10-951 2 8960,8964 C64.9, C80.9 8963 80 10-8041, 8050-8075,8082,812043 122, c64.9 813~8141,8143,8155,8190-8201,8210, 8211,8221-8231,8240,8241,8244-8246, 8260-8263,8290,8310,8320,8323,8401, 8430,8440,8480-8490,8504,8510,8550, 8560-8573 x312 80ocrs004 C64.9 764 KRAMAROVA AND STILLER APPENDIX - INTEKNATIONAL CLASSIFICATION OF CHILDHOOD CANCER (ICCC) (CONTINUED) ICD-0-2 codes Diagnostic group VII. YTpatic tumours a Hepatoblastoma b Hepatic carcinoma (c) Unspecified malignant hepatic tumours VIII. Malignant bone turnours (c) Ewing’s sarcoma (d) Other specified malignant bone tumours (e) Unspecified malignant bone tumours IX. Soft-tissue sarcomas (a) Rhabdomyosarcoma and embryonal sarcoma (b) Fibrosarcoma, neurofibrosdrcorna and other f i b r y a t o u s neoplasms c Ka osi’ssarcoma d Ot er specified soft-tissue sarcomas Moroholow Toooeraohv 8970 8010-8041,8050-8075,8082,81204122, c22.0, c22.1 8140,8141,8143,8155,8190-8201,8210, 8211,8230,8231,8240,8241,8244-8246, 8260-8263,8310,8320,8323,8401,8430. 8440,8480-8490,8504,8510,8550, 8560-8573 81604180 c22.0, c22.1 80004004 9180-9200 9220-9230 9231,9240 9260 9363,9364 8812,9250,9261-9330,9370 8000-8004,8800,8801,8803,8804 C40.0-C41.9 C40.GC41.9, C80.9 C40.O-C41.9 C40.0-C41.9 8900-8920,8991 8810,8811,8813-8833,9540-9561 ~ 9140 8840-8896.8982.8990.9040-9044.91209134,9150-9170,Y251,9581 8963 9231.9240.9363. 9364 9260’ (e) Unspecified soft-tissue sarcomas 8800-8804 COO.OX63.9, C65.9476.8 C00.0-C39.9, C47.oC80.9 COO.CLC39.9, C47.0476.8 C00.0-C39.9, C44.oC80.9 9060-91 022 C7O.M72.9, C75.1435.3 9060-9102 C00.0-C55.9, C57.0-C61.9, C63.O-C69.9, C73.9475.0, C75.4480.9 C56.9, C62.oC62.9 C56.9. C62.CC62.9 X. Germ-cell, trophoblastic and other gonadal neoplasms (a) lntracranial and intraspinal germ-cell turnours (b) Other and unspecified non-gonadal germcell turnours (c) Gonadal germ-cell turnours (d) Gonadal carcinomas (e) Other and unspecified malignant gonadal tumours XI. Carcinomas and other malignant epithelial (c) Nasopharyngeal carcinoma ( f ) Other and unspecified carcinomas 9060-9102 8010-8041,8050-8075, 8082, 8120-8122, 81304141,8143,8155,8190-8201,8210, 821 1,8221-8241,82444246,82604263, 8290.8310.8320.8323.8430.8440. 8480-8490; 8504; 8510; 8550; 8 5 d 8 5 7 3 8380,8381,8441-8473 8590-8670.9000 8000-8004 C56.9, C62.GC62.9 8370-8375 8010-8041,8050--8075,8082,81204122, c73.9 81304141,8155,8190,8200,8201,8211, 8230,8231,82444246,82604263,8290, 8310,8320,8323,8430,8440,8480,8481, 8500-8573 8330-8350 8010-8041,8050-8075,8082,81204122, C11.oC11.9 8130-8141,8155,8190,82OO, 8201,8211, 8230,8231,8244-8246,82604263,8290, 8310,8320,8323,8430.8440,8480,8481, 8504,8510,8550,8560-8573 8720-8780 8010-8041,8050-8075,8082-8110,8140, c44 .0-c44.9 8143,8147,8190,8200,8240,8246,8247, 8260,8310,8320,8323,8390-8420,8430, 8480,8542,85604573,8940 80104155,8190-8263,8290,8310,8314COO.oC10.9, C12.9-C21.8, 8323,8430-8440,8480-8580,8940,8941 C23.9-C39.9, C48.OX48.8, C50.0-C55.9. C57.O-C61.9. C63.0-C63.9; C65.9X72.9; C75.0-C80.9 765 CHILDHOOD CANCEK CLASSIFICATION APPENDIX - INTERSATIOKAL CLASSIFICATIONOF CHILDHOOD CANCEK (ICCC) (CONTINUED) ICD-0-2 codes Diagnoslic group XII. Other and unspecified malignant neoplasms (a) Other specified malignant tumours Morpholom 8930,8933,8950,8951,8971-8981,9020, 9050-9053. 9110. ~, 9580 800&8004 COO.O-C21.8, (23.9439.9, C42.0-C55.9. C57.0X61.9. C63.GC63.9;C65.9469.9; C73.9-C75.0,C75.4480.9 1 (b) Other unspecified malignant tumours ToDo.eraphy IBehaviour code / 1 is included in this rubric.JBehaviour codes /0 and /1 are included in this rubric.