{"orpha":314422,"name":"Ameloblastic carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare odontogenic tumor characterized by aggressive clinical course and local destruction, occurring in mandible more often than in maxilla. The most common symptom is a rapidly progressing painful swelling, but it may present as a benign cystic lesion or as a large, rapidly growing mass with ulceration, bone resorption and teeth mobility, as well. The tumor may metastasize, most commonly to the cervical lymph nodes and the lungs.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314422/","orphanet_url":"https://www.orpha.net/en/disease/detail/314422","names":{"fr":{"name":"Carcinome améloblastique","definition":"Tumeur odontogénique rare caractérisée par une évolution clinique agressive et une destruction locale affectant plus souvent la mandibule que le maxillaire. Le symptôme le plus courant est un oedème douloureux évoluant rapidement, mais la tumeur peut se présenter sous forme de lésion kystique bénigne ou de masse importante de croissance rapide avec ulcérations, résorption osseuse et mobilité dentaire. Elle peut former des métastases, le plus souvent dans les ganglions lymphatiques cervicaux et les poumons."},"de":{"name":"Karzinom, ameloblastisches"},"es":{"name":"Carcinoma ameloblástico","definition":"Es un tumor odontogénico poco frecuente caracterizado por un curso clínico agresivo y destrucción local, que se presenta en la mandíbula, con mayor frecuencia que en el maxilar. El síntoma más común es un edema doloroso que progresa rápidamente, pero se puede presentar como una lesión quística benigna o también como una masa grande de crecimiento rápido, con ulceración, reabsorción ósea y movilidad dental. El tumor puede metastatizar, más comúnmente a ganglios linfáticos cervicales y pulmones."},"it":{"name":"Carcinoma ameloblastico","definition":"Il carcinoma ameloblastico è una neoplasia odontogena rara, caratterizzata da un decorso clinico aggressivo, localmente distruttivo, che colpisce più spesso la mandibola rispetto alla mascella. Il sintomo più comune è la tumefazione dolorosa a progressione rapida, ma può esordire anche sotto forma di lesione cistica benigna, oppure come massa a crescita rapida, associata ad ulcerazione, riassorbimento osseo e mobilità dei denti. Il tumore può metastatizzare, di solito nei linfonodi cervicali e nei polmoni."},"nl":{"name":"Ameloblastisch carcinoom","definition":"Een zeldzame, odontogene tumor, die gekarakteriseerd wordt door een agressief klinisch verloop en lokale destructie, en die vaker voorkomt in de onderkaak dan in de bovenkaak. Het meest gangbare symptoom is een snel progressieve, pijnlijke zwelling, maar de tumor kan zich eveneens presenteren als een goedaardig cystisch letsel of als een grote, snelgroeiende massa met ulceratie, botresorptie en tandmobiliteit. De tumor kan metastaseren, het vaakst naar cervicale lymfeklieren en longen."},"pt":{"name":"Carcinoma ameloblástico"},"pl":{"name":"Rak ameloblastyczny","definition":"*Rak ameloblastyczny jest rzadkim guzem zębopochodnym, który charakteryzuje się agresywnym przebiegiem klinicznym i miejscową destrukcją, częściej dotyczącą żuchwy niż szczęki. Najczęstszym objawem jest szybko postępujący, bolesny obrzęk, ale guz może przybierać postać łagodnej zmiany torbielowatej lub dużej, szybko rosnącej masy z owrzodzeniem, resorpcją kości i przemieszczeniem zębów. Guz może dawać przerzuty, najczęściej do węzłów chłonnych szyjnych i płuc."},"cs":{"name":"Ameloblastický karcinom"},"uk":{"name":"Амелобластна карцинома","definition":"A rare odontogenic tumor characterized by aggressive clinical course and local destruction, occurring in mandible more often than in maxilla. The most common symptom is a rapidly progressing painful swelling, but it may present as a benign cystic lesion or as a large, rapidly growing mass with ulceration, bone resorption and teeth mobility, as well. The tumor may metastasize, most commonly to the cervical lymph nodes and the lungs."},"zh":{"name":"成釉细胞癌","definition":"一种罕见的牙源性肿瘤，特征为侵袭性临床表现和局部破坏，多发生于下颌骨而非上颌骨。最常见的症状是快速进展的疼痛性肿胀，但也可能表现为良性囊性病变或快速增长的大肿块，伴有溃疡、骨吸收和牙齿活动。肿瘤有转移可能，最常见部位是颈部淋巴结和肺部。"},"tr":{"name":"Ameloblastik karsinom"}},"xrefs":[{"source":"ICD-10","code":"C41.1","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2B5J","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"70175880"},{"source":"MONDO","code":"0006079","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1314678","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"11855","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10083863","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":314425,"name":"Rare odontogenic tumor","classifications":[197,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":40.0,"area":"Worldwide","status":"Validated","source":"22493395[PMID]_19674470[PMID]_23251064[PMID]_23099227[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}