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Int. J. Cancer:68,759-765 (1996)
8 1996 Wiley-Liss, Inc.
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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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PERCY,C., VAN HOLTEN,V. and MUIH,C. (eds.), Intemarional TSOKOS,M.,The diagnosis and classification of childhood rhabdomyoclassification of diseases for oncology, 2nd ed., World Health Organisa- sarcoma. Semin. diagnost. Pathol., 11,2638 (1994).
tion, Geneva (1992).
WABINGA,
H.R., PARKIN,
D.M., WABWIRE-MANGEN,
F. and MUGERWA,
RIVERA-LUNA,
R., ALTER-MOLCHADSKY,
N. CARIIENAS-CARDOS,
R. J.W., Cancer in Kampala, Uganda, in 1989-91: changes in incidence in
and MARTI~NEZ-GUERRA,
G., Langerhans cell histiocytosis in children
the era of AIDS. Int. J. Cancer; 54,2636 (1993).
under 2 years of age. Med. ped. Oncol., 26,334-343 (1996).
WRITISG
GROUP
OF THE IIISTIOCYTE
SOCIETY,
Histiocytosis syndromes
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L.E., Revision of in children. Lancet, I, 208-209 (1987).
the World Health Organisation Classification of Brain Tumors for ZUl.CH. K.J., IN C~OI.IAI3OKAI'IONWITH PATHOLOCISIS IN 14 COCXI'RIES,
childhood brain tumors. Cancer, 56,1869-1886 (1985).
Histological ty in8 of tumours of the central nervous system. International
SHIKAMIZU,
B.. BARRIGA,F., NEEOUAYE,
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FAVERA,
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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.
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