{"orpha":206538,"name":"Malignant non-dysgerminomatous germ cell tumor of ovary","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Non-dysgerminomatous germ cell cancer of ovary"],"definition":"Malignant non-dysgerminomatous germ cell tumor of ovary is a rare malignant germ cell tumor of ovary arising from germ cells in the ovary, frequently unilateral at diagnosis, usually presenting during adolescence with pelvic mass, fever, vaginal bleeding and acute abdomen, with certain subtypes being occasionally associated with isosexual precocity, virilization, hyperthyroidism or carcinoid syndrome. Histologically they comprise the following: embryonal carcinoma, Yolk sac tumor, polyembryoma and mixed germ cell tumor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-206538/","orphanet_url":"https://www.orpha.net/en/disease/detail/206538","names":{"fr":{"name":"Tumeur germinale maligne non dysgerminomateuse de l'ovaire","synonyms":["Cancer germinal non dysgerminomateux de l'ovaire"],"definition":"Tumeur maligne ovarienne germinale rare issue des cellules germinales de l'ovaire, souvent unilatérale au moment du diagnostic, se manifestant généralement pendant l'adolescence par une masse pelvienne, une fièvre, des saignements vaginaux et une symptomatologie abdominale aiguë. Certains sous-types peuvent occasionnellement être associés à une précocité isosexuelle, à une virilisation, à une hyperthyroïdie ou à un syndrome carcinoïde. Sur le plan histologique, on distingue : carcinome embryonnaire, tumeur du sac vitellin, polyembryome et tumeur germinale mixte."},"de":{"name":"Keimzelltumor des Ovars, nicht-dysgerminomatöser maligner","synonyms":["Nicht-dysgerminomatöser Keimzellkrebs des Ovars"]},"es":{"name":"Tumor maligno de células germinales no disgerminomatoso de ovario","synonyms":["Cáncer de células germinales no disgerminomatoso de ovario"],"definition":"Es un tumor maligno de células germinales del ovario poco frecuente que se origina en las células germinales del ovario. Suele manifestarse en la adolescencia, de forma unilateral en el momento del diagnóstico, como una masa pélvica, acompañada de fiebre, sangrado vaginal y abdomen agudo, asociándose ocasionalmente ciertos subtipos con precocidad isosexual, virilización, hipertiroidismo o síndrome carcinoide. Histológicamente comprende el carcinoma embrionario, el tumor del saco vitelino, el poliembrioma y el tumor mixto de células germinales."},"it":{"name":"Tumore non disgerminale maligno dell'ovaio a cellule germinali","synonyms":["Cancro non disgerminomatoso a cellule germinali","Tumore non disgerminale dell'ovaio a cellule germinali"]},"nl":{"name":"Maligne niet-dysgerminomateuze kiemceltumor van ovarium","synonyms":["Kwaadaardige niet-dysgerminomateuze kiemceltumor van eierstok","Niet-dysgerminomateuze kiemcelkanker van ovarium"],"definition":"Maligne niet-dysgerminomateuze kiemceltumor van het ovarium is een zeldzame maligne kiemceltumor van het ovarium (zie deze term) die ontstaat uit kiemcellen in het ovarium, vaak unilateraal voorkomt bij de diagnose, en zich manifesteert tijdens de adolescentie met een gezwel in het bekken, koorts, vaginale bloeding en acute buik. Sommige subtypes zijn occasioneel geassocieerd met <i>pubertas praecox</i> (voortijdige puberteit), virilisatie, hyperthyreoïdie of carcinoïdsyndroom (zie deze term). Histologisch omvatten ze embryonaal carcinoom, dooierzaktumor, polyembryoom en gemengde kiemceltumor."},"pt":{"name":"Tumor de células germinativas ováricas, não-disgerminoma"},"pl":{"name":"Malignant non-dysgerminomatous germ cell tumor of ovary","synonyms":["Non-dysgerminomatous germ cell cancer of ovary"]},"cs":{"name":"Maligní non-dysgerminomový tumor z germinálních buněk ovarií","synonyms":["Non-dysgerminomatózní nádor z germinálních buněk ovarií"]},"uk":{"name":"Злоякісна недисгерміноматозна герміногенна пухлина яєчника","synonyms":["Недисгерміноматозний герміногенний рак яєчника"],"definition":"Malignant non-dysgerminomatous germ cell tumor of ovary is a rare malignant germ cell tumor of ovary (see this term) arising from germ cells in the ovary, frequently unilateral at diagnosis, usually presenting during adolescence with pelvic mass, fever, vaginal bleeding and acute abdomen, with certain subtypes being occasionally associated with isosexual precocity, virilization, hyperthyroidism or carcinoid syndrome (see this term). Histologically they comprise the following: embryonal carcinoma, Yolk sac tumor, polyembryoma and mixed germ cell tumor."},"zh":{"name":"卵巢恶性非生殖细胞瘤","synonyms":["卵巢非无性细胞瘤性生殖细胞癌"],"definition":"卵巢恶性非无性细胞瘤生殖细胞瘤是一种罕见的卵巢恶性生殖细胞瘤（见该术语），起源于卵巢中的生殖细胞，一般累及单侧卵巢，常在青春期表现为盆腔肿块、发热、阴道出血和急腹症，少部分亚型与性早熟、男性化、甲状腺功能亢进或类癌综合征有关（见该术语）。组织学包括：胚胎癌、卵黄囊瘤、多胚瘤和混合生殖细胞瘤。"},"tr":{"name":"Habis over non-disgerminomatöz germ hücre tümörü","synonyms":["Yumurtalık disgerminomatöz olmayan germ hücreli kanser"]}},"xrefs":[{"source":"ICD-10","code":"C56","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C73.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1633883097"},{"source":"MONDO","code":"0016096","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5679783","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":35807,"name":"Malignant germ cell tumor of ovary","classifications":[202,205,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adolescent"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}