{"orpha":206489,"name":"Malignant germ cell tumor of the vagina","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Vaginal germ cell cancer","Vaginal germ cell malignant tumor"],"definition":"Malignant germ cell tumor of the vagina is an extremely rare, malignant, vulvovaginal neoplasm, deriving from primordial germ cells in the vagina, typically characterized by painless bloody vaginal discharge and a polypoid mass which protrudes from the vagina. Serum alpha-fetoprotein is usually elevated and rapid progression, local agression and early metastasis to liver and lungs is reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-206489/","orphanet_url":"https://www.orpha.net/en/disease/detail/206489","names":{"fr":{"name":"Tumeur germinale maligne du vagin","synonyms":["Cancer germinal du vagin"],"definition":"Tumeur vulvo-vaginale rare, dérivant des cellules germinales primordiales du vagin, caractérisée typiquement par des pertes vaginales sanglantes indolores et une masse polypoïde faisant saillie dans le vagin. Les taux sériques d'alpha-foetoprotéine sont généralement élevés ; une croissance tumorale rapide, une infiltration locale et des métastases précoces au foie et aux poumons ont été décrites."},"de":{"name":"Keimzelltumor der Vagina, maligner","synonyms":["Keimzellkrebs der Vagina","Vaginal-Keimzelltumor, maligner"]},"es":{"name":"Tumor maligno de células germinales de vagina","synonyms":["Cáncer de células germinales de vagina","Tumor vaginal de células germinales"],"definition":"El tumor maligno de células germinales de la vagina es una neoplasia vulvovaginal, maligna y muy poco frecuente, derivada de células germinales primordiales en la vagina. Está caracterizada por flujo vaginal sanguinolento no doloroso y una masa polipoide que protruye de la vagina. La alfa-fetoproteína sérica suele estar elevada y se ha descrito una progresión rápida, agresión local y metástasis temprana a hígado y pulmones."},"it":{"name":"Tumore vaginale maligno a cellule germinali","synonyms":["Cancro vaginale a cellule germinali"],"definition":"Il tumore vaginale maligno a cellule germinali è una neoplasia vulvo-vaginale maligna molto rara, derivata dalle cellule germinali primordiali della vagina. Di solito è caratterizzato da emorragie vaginali indolori e dalla presenza di una massa polipoide che protrude dalla vagina. In genere i livelli dell'alfa-fetoproteina nel siero sono elevati. Il tumore ha una progressione rapida ed è localmente aggressivo; si osservano metastasi precoci nel fegato e nei polmoni."},"nl":{"name":"Maligne kiemceltumor van vagina","synonyms":["Kwaadaardige vaginale kiemceltumor","Maligne vaginale kiemceltumor","Vaginale kiemcelkanker"],"definition":"Maligne kiemceltumor van de vagina is een uiterst zeldzaam, maligne, vulvovaginaal neoplasma, afgeleid van primordiale kiemcellen in de vagina, en wordt doorgaans gekarakteriseerd door pijnloze bloederige vaginale afscheiding en een polypoïde massa die uitsteekt vanuit de vagina. Het gehalte van alfa-foetoproteïne in het serum is meestal verhoogd en snelle progressie, locale agressie en vroege metastase naar lever en longen werden gerapporteerd."},"pt":{"name":"Tumor maligno vaginal de células germinativas"},"pl":{"name":"Złośliwy guz germinalny pochwy","synonyms":["Guz germinalny pochwy"],"definition":"*Złośliwy guz germinalny pochwy jest niezwykle rzadkim, złośliwym nowotworem sromu i pochwy, pochodzącym z pierwotnych komórek rozrodczych znajdujących się w pochwie; jest zazwyczaj bezbolesny, powoduje krwistą wydzieliną z pochwy i ma postać polipowatej masy wystającej z pochwy. Stężenie alfa-fetoproteiny w surowicy krwi jest zwykle podwyższone; opisywano szybki wzrost guza, miejscowe naciekanie i wczesne przerzuty do wątroby i płuc."},"cs":{"name":"Maligní nádor pochvy z germinálních buněk","synonyms":["Vaginální nádor z germinálních buněk"]},"uk":{"name":"Злоякісна герміногенна пухлина піхви","synonyms":["Вагінальний герміногенний рак","Піхвова герміногенна злоякісна пухлина"],"definition":"Malignant germ cell tumor of the vagina is an extremely rare, malignant, vulvovaginal neoplasm, deriving from primordial germ cells in the vagina, typically characterized by painless bloody vaginal discharge and a polypoid mass which protrudes from the vagina. Serum alpha-fetoprotein is usually elevated and rapid progression, local agression and early metastasis to liver and lungs is reported."},"zh":{"name":"阴道恶性生殖细胞瘤","synonyms":["阴道生殖细胞恶性肿瘤"],"definition":"阴道恶性生殖细胞瘤是一种极为罕见的恶性外阴阴道肿瘤，起源于阴道内原始生殖细胞，其主要临床表现为无痛性阴道血性分泌物和阴道息肉状肿块。血清甲胎蛋白通常升高，疾病进展迅速，侵犯周围组织，早期出现肝、肺转移瘤。"},"tr":{"name":"Vajinanın habis germ hücreli tümörü","synonyms":["Vajinal germ hücre kanseri","Vajinal germ hücreli habis tümör"]}},"xrefs":[{"source":"ICD-10","code":"C52","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C71.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1689464329"},{"source":"MONDO","code":"0016094","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4749402","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":180312,"name":"Rare vulvovaginal tumor","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}