{"orpha":254698,"name":"Epithelioid trophoblastic tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare gestational trophoblastic neoplasmcharacterized histologically by invasion of the myometrium and/or cervix uteri by regular epithelioid cells of chorion laeve type intermediate trophoblasts which clusters in a hyaline stroma. The disease generally occurs several years after pregnancy and indicative signs are irregular metrorrhagia and moderate increases in chorionic gonadotropin levels.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-254698/","orphanet_url":"https://www.orpha.net/en/disease/detail/254698","names":{"fr":{"name":"Tumeur trophoblastique épithélioïde","definition":"Tumeur trophoblastique gestationnelle rare caractérisée, sur le plan histologique, par l'invasion du myomètre et/ou du col de l'utérus par les cellules épithélioïdes régulières du trophoblaste intermédiaire de type chorionique, formant des amas dans un stroma hyalin. La maladie se déclare généralement plusieurs années après une grossesse, et les signes évocateurs sont des métrorragies irrégulières et une augmentation modérée des taux de gonadotrophine chorionique."},"de":{"name":"Trophoblasttumor, epitheloider","definition":"Eine seltene trophoblastische Neoplasie im Rahmen der Schwangerschaft, die histologisch durch eine Invasion des Myometriums und/oder der Zervix uteri durch regelmäßige epitheloide Zellen von intermediären Trophoblasten vom Typ Chorionlaeve gekennzeichnet ist, die sich in einem hyalinen Stroma ansammeln. Die Erkrankung tritt in der Regel mehrere Jahre nach der Schwangerschaft auf. Anzeichen dafür sind unregelmäßige Metrorrhagie und ein mäßiger Anstieg der Choriongonadotropinwerte."},"es":{"name":"Tumor trofoblástico epitelioide","definition":"Es una neoplasia trofoblástica gestacional poco frecuente caracterizada histológicamente por la invasión del miometrio y/o del cuello uterino por células epitelioides regulares de trofoblastos intermedios de tipo corion laeve que se agrupan en un estroma hialino. La enfermedad suele aparecer varios años después del embarazo y los signos indicativos son la metrorragia irregular y el aumento moderado de los niveles de gonadotropina coriónica."},"it":{"name":"Tumore trofoblastico epitelioide"},"nl":{"name":"Epithelioïde trofoblasttumor","definition":"Een zeldzaam gestationeel trofoblastisch neoplasma, histologisch gekenmerkt door invasie van myometrium en/of baarmoederhals door regelmatige epithelioïde cellen van intermediaire trofoblasten van het chorion laeve-type die zijn gebundeld in een hyalien stroma. De ziekte treedt doorgaans enkele jaren na zwangerschap op, en indicatieve verschijnselen zijn onregelmatige intermenstruele bloeding (metrorragie) en matige stijgingen van gehaltes van choriongonadotrofine."},"pt":{"name":"Tumor trofoblástico epitelióide","definition":"Neoplasia trofoblástica gestacional rara caracterizada histologicamente pela invasão do miométrio e/ou colo do útero por células epitelióides regulares de trofoblastos intermediários do tipo córion laeve que se agrupam num estroma hialino. A doença geralmente ocorre vários anos após a gravidez e os sinais são metrorragia irregular e aumentos moderados nos níveis de gonadotrofina coriónica."},"pl":{"name":"Epitelioidalny guz trofoblastyczny","definition":"Rzadki ciążowy nowotwór trofoblastyczny, który histologicznie charakteryzuje się naciekaniem myometrium i/lub szyjki macicy przez regularne komórki epitelioidalne trofoblastu pośredniego typu kosmówkowego, które skupiają się w zrębie szklistym. Choroba zwykle rozwija się kilka lat po ciąży, a jej objawami są nieregularne krwotoki maciczne i umiarkowany wzrost poziomu gonadotropiny kosmówkowej."},"cs":{"name":"Epiteloidní trofoblastický nádor"},"uk":{"name":"Епітеліоїдна трофобластична пухлина","definition":"A rare gestational trophoblastic neoplasmcharacterized histologically by invasion of the myometrium and/or cervix uteri by regular epithelioid cells of chorion laeve type intermediate trophoblasts which clusters in a hyaline stroma. The disease generally occurs several years after pregnancy and indicative signs are irregular metrorrhagia and moderate increases in chorionic gonadotropin levels."},"zh":{"name":"上皮样滋养层细胞肿瘤","definition":"上皮样滋养细胞肿瘤是一种极其罕见的妊娠滋养细胞肿瘤(GTT；见该词条)，通常发生在妊娠数年后。"},"tr":{"name":"Epiteloid trofoblastik tümör"}},"xrefs":[{"source":"ICD-10","code":"C58","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C75.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1836453605"},{"source":"ICD-11","code":"XH8FW3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"751729083"},{"source":"MONDO","code":"0016787","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1266159","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":59305,"name":"Gestational trophoblastic neoplasm","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"ORPHANET"},{"type":"Lifetime Prevalence","qualification":"Class only","class":null,"value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}