raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:55880

Chondrosarcoma

Type
Disease
Group
Disorder
Inheritance
Not applicable, Unknown
Age of onset
Adult
Status
active

Chondrosarcoma is a malignant bone tumor arising from cartilaginous tissue, most frequently occuring at the ends of the femur and tibia, the proximal end of the humerus and the pelvis; and presenting with a palpable mass and progressive pain. Chondrosarcoma is usually slow growing at low histological grades and can be well managed by intralesional curettage or en-block wide resection.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enChondrosarcoma
frChondrosarcome
deChondrosarkom
esCondrosarcoma
itCondrosarcoma
nlChondrosarcoom
ptCondrosarcoma
plChrzęstniakomięsak
csChondrosarkom
ukХондросаркома
zh软骨肉瘤
trKondrosarkom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM215300Evalidated
ICD-10C49.9NTBTattributedvalidated
ICD-112B50Especificvalidated
MONDO0008977Evalidated
MeSHD002813Evalidated
UMLSC0008479Evalidated
GARD6055Evalidated
MedDRA10008734Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

1 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
EXT1Candidate gene tested inNot yet assessedHGNC:3512ENSG000001821976081778q24.11exostosin glycosyltransferase 1
5

Prevalence and incidence

3 records
TypeClassValueAreaQualificationValidationSource
Annual incidence1-9 / 1 000 0000.24 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Point prevalence1-9 / 100 000—EuropeClass onlyValidated22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]
Lifetime Prevalence1-9 / 100 0003.55 / 100 000EuropeValue and classValidated22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card