{"orpha":141067,"name":"Cervicofacial fibrochondroma","type":"Morphological anomaly","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare extraskeletal chondroma located in the head and neck region, histologically typically characterized by lobules of mature, adult hyaline cartilage with chondrocytic cells identifiable in lacunae, and prominent fibrosis. Malignant transformation has not been described.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-141067/","orphanet_url":"https://www.orpha.net/en/disease/detail/141067","names":{"fr":{"name":"Fibrochondrome cervico-facial","definition":"Chondrome extrasquelettique rare situé dans la région de la tête et du cou, caractérisé histologiquement par des lobules de cartilage hyalin mature, adulte, avec des chondrocytes identifiables dans les lacunes, et une importante fibrose. Aucune transformation maligne n'a été décrite."},"de":{"name":"Fibrochondrom, zervikofaziales","definition":"Ein seltenes extraskelettales Chondrom im Kopf- und Halsbereich, histologisch typischerweise gekennzeichnet durch Läppchen aus reifem, erwachsenem hyalinem Knorpel mit in Lakunen erkennbaren chondrozytären Zellen und ausgeprägter Fibrose. Eine maligne Transformation ist nicht beschrieben worden."},"es":{"name":"Fibrocondroma cervicofacial","definition":"Es un condroma extra-esquelético poco frecuente localizado en la región de la cabeza y del cuello. Por lo general está caracterizado histológicamente por lóbulos de cartílago hialino maduro con condrocitos localizados en lagunas y por destacada fibrosis. No se ha descrito la transformación maligna."},"it":{"name":"Fibrocondroma cervico-facciale","definition":"Si tratta di un condroma extrascheletrico raro, localizzato sulla testa e sul collo, caratterizzato, dal punto di vista istologico, da lobuli di cartilagine ialina matura adulta, con condrociti localizzati nelle lacune ed evidente fibrosi. Non si associa ad una evoluzione maligna."},"nl":{"name":"Cervicofaciaal fibrochondroom","definition":"Een zeldzaam, extraskeletaal chondroom gelokaliseerd ter hoogte van hoofd en nek, histologisch doorgaans gekarakteriseerd door lobuli bestaande uit matuur, volwassen hyalien kraakbeen met chondrocyten gesitueerd in holten, en aanzienlijke fibrose. Maligne transformatie werd niet beschreven."},"pt":{"name":"Fibrocondroma cervicofacial"},"pl":{"name":"Włókniakochrzęstniak szyi i twarzy","synonyms":["Chrzęstniak śródkostny szyjno-twarzowy"]},"cs":{"name":"Cervikofaciální fibrochondrom","synonyms":["Cervikofaciální enchondrom"]},"uk":{"name":"Шийно-лицьова фіброхондрома","definition":"A rare extraskeletal chondroma located in the head and neck region, histologically typically characterized by lobules of mature, adult hyaline cartilage with chondrocytic cells identifiable in lacunae, and prominent fibrosis. Malignant transformation has not been described."},"zh":{"name":"颈面部纤维软骨瘤","definition":"一种罕见的骨外软骨瘤，位于头颈部，其组织学特征是小叶成熟，成人透明软骨，伴可见于陷窝内的软骨细胞和明显纤维化。尚未见恶性转化。"},"tr":{"name":"Servikofasiyal fibrokondrom"}},"xrefs":[{"source":"ICD-10","code":"D21.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"LA6Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"427530222"},{"source":"MONDO","code":"0015383","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5231314","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"19941","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68329,"name":"Rare maxillo-facial surgical disease"},"parents":[{"orpha":155835,"name":"Cysts and fistulae of the face and oral cavity","classifications":[147,200]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}