{"orpha":447777,"name":"Keratocystic odontogenic tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["KTOC","Odontogenic keratocystoma"],"definition":"A rare odontogenic tumor characterized by an unilocular or multilocular cyst most commonly located in the posterior body and lower ramus of the mandible, often surrounding the crown of the third molar. Histopathologically, the lesion shows a lining of parakeratinized stratified squamous epithelium with palisading hyperchromatic basal cells. Patients may be asymptomatic or present with local infection and/or signs and symptoms of mass effect. Recurrence is rare after complete surgical removal. Some patients have multiple cysts (metachronous or synchronous), especially in the context of nevoid basal cell carcinoma syndrome (Gorlin syndrome).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-447777/","orphanet_url":"https://www.orpha.net/en/disease/detail/447777","names":{"fr":{"name":"Tumeur odontogéne kératokystique","synonyms":["KTOC","Kératokyste odontogène"],"definition":"Tumeur odontogénique rare caractérisée par un kyste uniloculaire ou multiloculaire situé le plus souvent dans la partie postérieure et inférieure de la branche de la mandibule, entourant souvent la couronne de la troisième molaire. L'examen histopathologique révèle une lésion présentant un épithélium de revêtement d'aspect pavimenteux stratifié, parakératinisé, avec des cellules basales hyperchromatiques en disposition palissadique. Les patients peuvent être asymptomatiques ou présenter une infection locale et/ou des signes cliniques et symptômes d'effet de masse. La récidive est rare après une ablation chirurgicale complète. Certains patients présentent des kystes multiples (métachrones ou synchrones), notamment dans le cadre du syndrome de carcinome basocellulaire névoïde (syndrome de Gorlin)."},"de":{"name":"Keratozystischer odontogener Tumor","synonyms":["KTOC","Keratozyste, odontogene"],"definition":"Ein seltener odontogener Tumor, der durch eine unilokulare oder multilokulare Zyste gekennzeichnet ist, die sich meist im hinteren Körper und im unteren Ramus des Unterkiefers befindet und häufig die Krone des dritten Molaren umgibt. Histopathologisch zeigt die Läsion eine Auskleidung mit parakeratinisiertem, geschichtetem Plattenepithel mit palisierenden hyperchromatischen Basalzellen. Die Patienten können asymptomatisch sein oder eine lokale Infektion und/oder Anzeichen und Symptome einer Gewebeverdichtung aufweisen. Nach vollständiger chirurgischer Entfernung ist ein Rezidiv selten. Einige Patienten haben mehrere Zysten (metachron oder synchron), insbesondere im Zusammenhang mit dem nevoiden Basalzellkarzinomsyndrom (Gorlin-Syndrom)."},"es":{"name":"Tumor odontogénico queratoquístico","synonyms":["KTOC","Queratocistoma odontogénico","Queratoquiste odontogénico"],"definition":"Es un tumor odontogénico poco frecuente caracterizado por un quiste uni- o multilocular localizado con mayor frecuencia en la parte posterior del cuerpo y en la rama mandibular inferior, a menudo alrededor de la corona del tercer molar. Histopatológicamente, la lesión muestra un revestimiento de epitelio escamoso estratificado paraqueratinizado con células basales hipercromáticas en empalizada. Los pacientes pueden permanecer asintomáticos o presentarse con una infección local y/o signos y síntomas asociados al efecto masa. La recurrencia es poco frecuente después de la resección quirúrgica completa. Algunos pacientes presentan múltiples quistes (metacrónicos o sincrónicos), especialmente en el contexto del síndrome de carcinoma nevoide de células basales (síndrome de Gorlin)."},"it":{"name":"Tumore cheratocistico odontogeno","synonyms":["Cheratocisti odontogena","KTOC"],"definition":"Si tratta di un tumore odontogeno raro, caratterizzato da una cisti uniloculare o multiloculare, di solito localizzata nel corpo posteriore e nel ramo inferiore della mandibola, e che spesso circonda la corona del terzo molare. Dal punto di vista istopatologico, la lesione presenta un rivestimento di epitelio squamoso stratificato paracheratinizzato con cellule basali ipercromatiche a palizzata. I pazienti possono essere asintomatici o presentare un'infezione locale e/o i sintomi correlati alla presenza di una massa. Dopo la rimozione chirurgica completa, le recidive sono rare. Alcuni pazienti presentano cisti multiple (che insorgono simultaneamente o in successione), soprattutto nel quadro della sindrome del carcinoma nevoide delle cellule basali (sindrome di Gorlin)."},"nl":{"name":"Keratocystische odontogene tumor","synonyms":["KTOC","Odontogeen keratocystoom"],"definition":"Een zeldzame odontogene tumor, gekarakteriseerd door een uniloculaire of multiloculaire cyste doorgaans gesitueerd in posterieure corpus en onderste ramus van mandibula, vaak rond de kroon van de derde molaar. Histopathologisch is de laesie bekleed met geparakeratiniseerd gelaagd plaveiselepitheel met gepalissadeerde hyperchromatische basale cellen. Patiënten kunnen asymptomatisch of zich presenteren met lokale infectie en/of verschijnselen en symptomen van massa-effect. Recidief is zeldzaam na volledige chirurgische verwijdering. Sommige patiënten hebben meerdere cysten (metachroon of synchroon), vooral in de context van basaalcelnaevus-syndroom (syndroom van Gorlin)."},"pt":{"name":"Tumor odontogénico queratoquístico"},"pl":{"name":"Guz zębopochodny rogowaciejący","synonyms":["Zębopochodny guz keratocystyczny"]},"cs":{"name":"Keratocystický odontogenní tumor","synonyms":["KTOC","Odontogenní keratocysta"]},"uk":{"name":"Кератокістозна одонтогенна пухлина","synonyms":["KTOC","Одонтогенна кератоцистома"],"definition":"A rare odontogenic tumor characterized by an unilocular or multilocular cyst most commonly located in the posterior body and lower ramus of the mandible, often surrounding the crown of the third molar. Histopathologically, the lesion shows a lining of parakeratinized stratified squamous epithelium with palisading hyperchromatic basal cells. Patients may be asymptomatic or present with local infection and/or signs and symptoms of mass effect. Recurrence is rare after complete surgical removal. Some patients have multiple cysts (metachronous or synchronous), especially in the context of nevoid basal cell carcinoma syndrome (Gorlin syndrome)."},"zh":{"name":"角化囊性牙源性肿瘤","synonyms":["牙源性角化囊肿"]},"tr":{"name":"Keratokistik odontojenik tümör","synonyms":["KTOC","Odontojenik keratokistoma"]}},"xrefs":[{"source":"ICD-10","code":"D16.4","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2E83.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"526694398"},{"source":"ICD-11","code":"2E83.Z","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1523661015"},{"source":"MONDO","code":"0018648","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1708604","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98026,"name":"Rare odontologic disease"},"parents":[{"orpha":314425,"name":"Rare odontogenic tumor","classifications":[197,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}