{"orpha":180234,"name":"Mixed germ cell tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare germ cell tumor characterized by composition of two or more malignant germ cell components, the most common combination being dysgerminoma and yolk sac tumor. The tumors typically occur between childhood and young adulthood. They are usually located in the gonads, occasionally also in other regions. Clinical presentation corresponds to the individual germ cell components and the tumor location; manifestations may include abdominal pain, abdominal mass, and menstrual disorder in females, and a testicular mass in males. The most important prognostic factor is tumor stage.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-180234/","orphanet_url":"https://www.orpha.net/en/disease/detail/180234","names":{"fr":{"name":"Tumeur germinale maligne mixte","definition":"Tumeur germinale rare, caractérisée par au moins deux types de cellules germinales malignes, l'association la plus fréquente étant dysgerminome et tumeur du sac vitellin. Les tumeurs surviennent généralement chez l'enfant et chez le jeune adulte. Elles sont habituellement localisées dans les gonades et, plus occasionnellement, dans d'autres régions du corps. La présentation clinique dépend des types de cellules germinales et de l'emplacement de la tumeur, mais peut comprendre douleurs abdominales, masse abdominale et troubles du cycle menstruel chez la femme, ainsi que masse testiculaire chez l'homme. Le facteur pronostique le plus important est le stade de la tumeur."},"de":{"name":"Keimzelltumor, gemischter","definition":"Ein seltener Keimzelltumor, der durch die Zusammensetzung von zwei oder mehr malignen Keimzellkomponenten gekennzeichnet ist, wobei die häufigste Kombination Dysgerminom und Dottersacktumor ist. Die Tumoren treten typischerweise zwischen der Kindheit und dem jungen Erwachsenenalter auf. Sie sind meist in den Keimdrüsen lokalisiert, gelegentlich auch in anderen Regionen. Das klinische Erscheinungsbild richtet sich nach den einzelnen Keimzellkomponenten und der Tumorlokalisation; es kann sich bei Frauen durch Unterleibsschmerzen, abdominelle Gewebeverdichtung und Menstruationsstörungen und bei Männern durch eine Hodenverdichtung äußern. Der wichtigste prognostische Faktor ist das Tumorstadium."},"es":{"name":"Tumor mixto de células germinales","definition":"Es un tumor de células germinales poco frecuente caracterizado por la presencia de dos o más componentes de células germinales malignas, siendo la combinación más frecuente el disgerminoma y el tumor del saco vitelino. Los tumores aparecen típicamente entre la infancia y la edad adulta temprana. Por lo general, se localizan en las gónadas y, ocasionalmente, en otras regiones. La presentación clínica depende de los componentes individuales de las células germinales y la localizarión del tumor; las manifestaciones pueden incluir dolor abdominal, masa abdominal y trastornos menstruales en mujeres, y una masa testicular en varones. El factor pronóstico más importante es el estadío del tumor."},"it":{"name":"Tumore a cellule germinali miste","definition":"Si tratta di un tumore raro a cellule germinali, caratterizzato da due o più componenti maligne di cellule germinali; l'associazione più comune è tra disgerminoma e il tumore del sacco vitellino. In genere le neoplasie esordiscono tra l'infanzia e la prima età adulta; si localizzano nelle gonadi e solo occasionalmente in altre regioni. Il quadro clinico dipende dalle componenti a cellule germinali e dalla localizzazione del tumore, e comprende il dolore addominale, la presenza di una massa addominale e le anomalie del ciclo mestruale nelle femmine, e la presenza di una massa testicolare nei maschi. Il fattore prognostico più importante è lo stadio del tumore."},"nl":{"name":"Gemengde kiemceltumor","definition":"Een zeldzame kiemceltumor, gekarakteriseerd door een samenstelling uit twee of meer componenten ontstaan uit maligne kiemcellen, met als meest gangbare combinatie dysgerminoom en dooierzaktumor. De tumoren treden doorgaans op tussen de kindertijd en jonge volwassenheid. Meestal zijn ze gesitueerd in de gonaden, maar soms ook in andere lichaamsdelen. De klinische presentatie stemt overeen met de individuele componenten afkomstig van kiemcellen en de locatie van de tumor; mogelijke manifestaties zijn onder meer abdominale pijn, abdominale massa, en menstruatiestoornis bij vrouwen, en een massa van testis bij mannen. Het stadium van de tumor is de belangrijkste prognostische factor."},"pt":{"name":"Tumor misto de células germinativas"},"pl":{"name":"Mieszany guz germinalny"},"cs":{"name":"Smíšený germinální nádor"},"uk":{"name":"Змішана герміногенна пухлина","definition":"A rare germ cell tumor characterized by composition of two or more malignant germ cell components, the most common combination being dysgerminoma and yolk sac tumor. The tumors typically occur between childhood and young adulthood. They are usually located in the gonads, occasionally also in other regions. Clinical presentation corresponds to the individual germ cell components and the tumor location; manifestations may include abdominal pain, abdominal mass, and menstrual disorder in females, and a testicular mass in males. The most important prognostic factor is tumor stage."},"zh":{"name":"混合性生殖细胞瘤"},"tr":{"name":"Karma germ hücreli tümör"}},"xrefs":[{"source":"ICD-10","code":"C80.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2D4Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"618729503"},{"source":"ICD-11","code":"XH2PS1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1277295600"},{"source":"MONDO","code":"0015864","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334524","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":252021,"name":"Mixed germ cell tumor of central nervous system","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.01,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}