{"orpha":314419,"name":"Ameloblastoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, benign, slow-growing odontologic tumor located in the mandible, and on occasion the maxilla, characterized by painless, variable-sized jaw swelling, which if left untreated may lead to a grotesque facial appearance. Occasionally, paresthesias, tooth displacement and adjacent root resorption may be associated. Local invasion is frequently observed, but malignant transformation and metastasis are not common.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314419/","orphanet_url":"https://www.orpha.net/en/disease/detail/314419","names":{"fr":{"name":"Améloblastome","definition":"L'améloblastome est une tumeur odontologique rare et bénigne, progressant lentement, située au niveau de la mandibule et parfois du maxillaire. Il se caractérise par un gonflement indolore de la mâchoire, de taille variable, qui peut conduire à une apparence grotesque du visage en l'absence de traitement. A l'occasion, une paresthésie, un déplacement dentaire et une résorption de la racine adjacente peuvent être associés. Une invasion locale est fréquemment observée, mais la transformation maligne et les métastases sont rares."},"de":{"name":"Ameloblastom","definition":"Ein seltener, gutartiger, langsam wachsender odontologischer Tumor, der im Unterkiefer und gelegentlich auch im Oberkiefer lokalisiert ist und durch schmerzlose, unterschiedlich große Kieferschwellungen gekennzeichnet ist, die unbehandelt zu einem grotesken Gesichtsausdruck führen können. Gelegentlich können Parästhesien, Zahnverschiebungen und benachbarte Wurzelresorption auftreten. Eine lokale Invasion wird häufig beobachtet, aber eine maligne Transformation und Metastasierung sind nicht häufig."},"es":{"name":"Ameloblastoma","synonyms":["Ameloblastoma de la mandíbula"],"definition":"Es un tumor odontogénico benigno y poco frecuente de crecimiento lento, localizado en la mandíbula y, ocasionalmente, en el maxilar superior, caracterizado por la inflamación indolora y de tamaño variable de la mandíbula, que sin tratamiento puede dar lugar a una apariencia facial grotesca. Ocasionalmente, puede estar asociado a parestesias, desplazamiento de los dientes y reabsorción de la raíz adyacente. Con frecuencia se observa invasión local, aunque la transformación maligna y la metástasis no son comunes."},"it":{"name":"Ameloblastoma","definition":"L'ameloblastoma è un tumore odontogeno raro e benigno a crescita lenta, localizzato nella mandibola e, raramente, nella mascella. È caratterizzato da un gonfiore della mascella/mandibola di ampiezza variabile, che, se non trattato, può esitare in una facies grottesca. Raramente sono presenti parestesie, dislocazione dei denti e riassorbimento delle radici adiacenti. Spesso ha una disseminazione locale, mentre l'evoluzione maligna e le metastasi sono rare."},"nl":{"name":"Ameloblastoom","synonyms":["Ameloblastoom van de kaak"],"definition":"Een zeldzame, goedaardige, traaggroeiende, odontologische tumor gesitueerd in de onderkaak, en in sommige gevallen in de bovenkaak, gekarakteriseerd door een pijnloze en in omvang variabele zwelling van de kaak die zonder behandeling kan leiden tot een grotesk uiterlijk van het gelaat. Occasioneel kunnen paresthesie, verplaatsing van tanden en resorptie van aangrenzende wortels geassocieerd zijn. Lokale invasie wordt geregeld waargenomen, maar maligne transformatie en metastase komen niet veel voor."},"pt":{"name":"Ameloblastoma"},"pl":{"name":"Ameloblastoma"},"cs":{"name":"Ameloblastom"},"uk":{"name":"Амелобластома","definition":"A rare, benign, slow-growing odontologic tumor located in the mandible, and on occasion the maxilla, characterized by painless, variable-sized jaw swelling, which if left untreated may lead to a grotesque facial appearance. Occasionally, paresthesias, tooth displacement and adjacent root resorption may be associated. Local invasion is frequently observed, but malignant transformation and metastasis are not common."},"zh":{"name":"成釉细胞瘤","definition":"一种罕见的良性、生长缓慢的牙髓肿瘤，位于下颌骨，有时发生在上颌骨，其特征是无痛、大小不等的颌骨肿胀，如果不治疗，可能会导致面部畸形。偶尔可表现为感觉异常、牙齿移位和邻近牙根吸收。常见局部侵袭，但恶性转化和转移少见。"},"tr":{"name":"Ameloblastom"}},"xrefs":[{"source":"ICD-10","code":"D16.4","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-10","code":"D16.5","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2B5J","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"268617138"},{"source":"ICD-11","code":"2E83.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1367271306"},{"source":"ICD-11","code":"XH1SV4","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"92385094"},{"source":"ICD-11","code":"XH96J9","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"2140324669"},{"source":"MONDO","code":"0017795","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000564","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0002448","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5747","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10066796","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":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}