{"orpha":55880,"name":"Chondrosarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-55880/","orphanet_url":"https://www.orpha.net/en/disease/detail/55880","names":{"fr":{"name":"Chondrosarcome","definition":"Tumeur osseuse maligne prenant naissance dans le tissu cartilagineux, localisée le plus souvent aux extrémités du fémur et du tibia, à l'extrémité proximale de l'humérus et du bassin, se manifestant par une masse palpable et une douleur progressive. En général, un chondrosarcome de bas grade se développe lentement et se traite bien par un curetage intralésionnel ou une résection large en un seul bloc."},"de":{"name":"Chondrosarkom","definition":"Das Chondrosarkom ist ein bösartiger Knochentumor, der aus Knorpelgewebe entsteht. Es tritt vorwiegend an den Enden der Oberschenkelknochen und Schienbeine, dem proximalen Ende des Oberarmknochens sowie im Becken auf und manifestiert mit einer tastbaren Masse und fortschreitenden Schmerzen. Das Chondrosarkom wächst in der Regel langsam, zeigt histologisch zumeist eine gute Differenzierung und kann durch intralesionale Kürettage oder en-blockweite Resektion gut beherrscht werden."},"es":{"name":"Condrosarcoma","definition":"Es un tumor óseo maligno que se origina en el tejido cartilaginoso y se presenta con mayor frecuencia en los extremos del fémur y la tibia, el extremo proximal del húmero y la pelvis. Se presenta como una masa palpable acompañada de dolor progresivo. El condrosarcoma suele tener un crecimiento lento en los grados histológicos bajos y puede manejarse bien con curetaje intralesional o resección extensa en bloque."},"it":{"name":"Condrosarcoma","definition":"Il condrosarcoma è un tumore osseo maligno che origina dal tessuto cartilagineo; nella maggior parte dei casi si localizza alle estremità del femore e della tibia e all'estremità prossimale dell'omero e del bacino; esordisce con una massa palpabile e con dolore progressivo. Di solito, il condrosarcoma cresce lentamente e presenta un basso grado di malignità, perciò può essere gestito con una levigatura intralesionale o con un'ampia resezione en bloc."},"nl":{"name":"Chondrosarcoom","definition":"Chondrosarcoom is een kwaadaardige bottumor die ontstaat uit kraakbeenweefsel, die meestal voorkomt aan het uiteinde van het dijbeen (femur) en het scheenbeen (tibia), het proximale uiteinde van het opperarmbeen (humerus) en het bekken (pelvis), en die zich uit met een tastbare massa en progressieve pijn. Chondrosarcoom groeit meestal traag met een lage histologische graad en valt goed te beheersen door intralaesionale curettage of ruime en bloc resectie."},"pt":{"name":"Condrosarcoma"},"pl":{"name":"Chrzęstniakomięsak"},"cs":{"name":"Chondrosarkom"},"uk":{"name":"Хондросаркома","definition":"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."},"zh":{"name":"软骨肉瘤"},"tr":{"name":"Kondrosarkom"}},"xrefs":[{"source":"OMIM","code":"215300","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B50","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1625755389"},{"source":"MONDO","code":"0008977","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D002813","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0008479","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6055","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10008734","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":223727,"name":"Bone sarcoma","classifications":[199,202]}],"children":[],"genes":[{"symbol":"EXT1","name":"exostosin glycosyltransferase 1","gene_type":"gene with protein product","association":"Candidate gene tested in","status":"Not yet assessed","source":null,"hgnc":"3512","ensembl":"ENSG00000182197","omim":"608177","loci":["8q24.11"]}],"genes_via_subtypes":[],"inheritance":["Not applicable","Unknown"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.24,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.55,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}