{"orpha":883,"name":"Extragonadal teratoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Extragonadal teratoma is an extremely rare, benign or malignant germ cell tumor characterized, clinically, by a teratoma presenting in an extragonadal location (e.g. retroperitoneum, mediastinum, craniofacial or sacrococcygeal region, intraosseous, solid organs) and, histologically, by displaying well-differentiated structures, as well as immature elements. Presenting symptoms are variable depending on size and location of tumor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-883/","orphanet_url":"https://www.orpha.net/en/disease/detail/883","names":{"fr":{"name":"Tératome extragonadique","definition":"Tumeur germinale rare, bénigne ou maligne, caractérisée sur le plan clinique par un tératome extragonadique (localisé, par exemple, dans le rétropéritoine, le médiastin, la région craniofaciale ou sacrococcygienne, intra-osseuse, dans les organes solides) et, sur le plan histologique, par la présence de structures bien différenciées, ainsi que d'éléments immatures. Les symptômes dépendent de la taille et de la localisation de la tumeur."},"de":{"name":"Teratom, extragonadales","definition":"Das extragonadale Teratom ist ein extrem seltener, gutartiger oder bösartiger Keimzelltumor, der klinisch durch ein Teratom in extragonadaler Lage (z. B. Retroperitoneum, Mediastinum, kraniofaziale oder sakrokokzygeale Bereiche, intraossär, solide Organe) und histologisch durch das Vorhandensein gut differenzierter Strukturen sowie unreifer Elemente gekennzeichnet ist. Die Symptome sind je nach Größe und Lage des Tumors unterschiedlich."},"es":{"name":"Teratoma extragonadal","definition":"Es un tumor de células germinales benigno o maligno muy poco frecuente, caracterizado clínicamente por su presentación en una localización extragonadal (p. ej., retroperitoneo, mediastino, región craneofacial o sacrococcígea, intraósea, órganos sólidos) e histológicamente, por la presencia de estructuras bien diferenciadas, así como de elementos inmaduros. Los síntomas de presentación varían según el tamaño y la ubicación del tumor."},"it":{"name":"Teratoma extragonadico","definition":"Il teratoma extragonadico è una neoplasia molto rara, benigna o maligna, delle cellule germinali, caratterizzata dal punto di vista clinico da un teratoma con localizzazione extragonadica (a livello del retroperitoneo, del mediastino, della regione craniofacciale, sacrococcigea oppure intraossea o degli organi solidi). Dal punto di vista istologico, presenta strutture ben differenziate con elementi immaturi. I sintomi d'esordio sono variabili e dipendono dalle dimensioni e dalla localizzazione del tumore."},"nl":{"name":"Extragonadaal teratoom","definition":"Extragonadaal teratoom is een uiterst zeldzame, goed- of kwaadaardige kiemceltumor die klinisch gekarakteriseerd wordt door een teratoom dat voorkomt op een extragonadale locatie (e.g. retroperitoneum, mediastinum, craniofaciale of sacrococcygeale regio, intraossaal, solide organen), en die histologisch gekenmerkt wordt door het vertonen van goed gedifferentieerde structuren, alsook onvolgroeide elementen. De symptomen die voorkomen, variëren afhankelijk van de omvang en de locatie van de tumor."},"pt":{"name":"Teratoma extragonadal"},"pl":{"name":"Potworniak zewnątrzgonadalny","definition":"*Potworniak zewnątrzgonadalny jest niezwykle rzadkim, łagodnym lub złośliwym guzem zarodkowym, który przyjmuje kliniczną postać potworniaka w lokalizacji poza gonadami (np. w przestrzeni zaotrzewnowej, śródpiersiu, w okolicy twarzoczaszki lub kości krzyżowej, wewnątrz kości, w narządach wewnętrznych). W budowie histologicznej można wyodrębnić dobrze zróżnicowane struktury, jak również elementy niedojrzałe. Objawy zależą od wielkości i umiejscowienia guza."},"cs":{"name":"Extragonadální teratom"},"uk":{"name":"Екстрагонадна тератома","definition":"Extragonadal teratoma is an extremely rare, benign or malignant germ cell tumor characterized, clinically, by a teratoma presenting in an extragonadal location (e.g. retroperitoneum, mediastinum, craniofacial or sacrococcygeal region, intraosseous, solid organs) and, histologically, by displaying well-differentiated structures, as well as immature elements. Presenting symptoms are variable depending on size and location of tumor."},"zh":{"name":"性腺外畸胎瘤"},"tr":{"name":"Ekstragonadal teratom"}},"xrefs":[{"source":"ICD-10","code":"D48.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0019500","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D013724","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4708601","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10043276","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":99913,"name":"Extragonadal non-dysgerminomatous germ cell tumor","classifications":[202,233]}],"children":[{"orpha":141077,"name":"Epignathus","type":"Clinical subtype"},{"orpha":141107,"name":"Nasopharyngeal teratoma","type":"Clinical subtype"},{"orpha":494421,"name":"Sacrococcygeal teratoma","type":"Clinical subtype"},{"orpha":252018,"name":"Teratoma of the central nervous system","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}