{"orpha":363494,"name":"Non-seminomatous germ cell tumor of testis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Non-dysgerminomatous germ cell tumor of testis","Testicular non seminomatous germ cell tumor","Testicular non-dysgerminomatous germ cell tumor"],"definition":"A form of testicular germ cell tumor occurring in the third decade of life with a usually painless unilateral mass in the scrotum or, in some cases, with gynaecomastia and/or back and flack pain. The clinical course is more aggressive than testicular seminomatous germ cell tumors with rapid involvement of blood vessels and a poorer prognosis. Histologically, the tumour can be either undifferentiated (embryonal carcinoma), differentiated (teratoma, yolk sac tumor, choriocarcinoma), or can consist of a mixture of seminomatous and nonseminomatous components.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-363494/","orphanet_url":"https://www.orpha.net/en/disease/detail/363494","names":{"fr":{"name":"Tumeur germinale non séminomateuse des testicules","synonyms":["Tumeur germinale non dysgerminomateuse des testicules","Tumeur testiculaire germinale non dysgerminomateuse","Tumeur testiculaire germinale non-séminomateuse"],"definition":"Forme de tumeur germinale des testicules se manifestant au cours de la troisième décennie de la vie par une masse unilatérale généralement indolore dans le scrotum ou, dans certains cas, par une gynécomastie et/ou des douleurs au dos et au flanc. L'évolution clinique est plus agressive que celle des tumeurs germinales séminomateuses des testicules, l'atteinte des vaisseaux sanguins est rapide et le pronostic plus défavorable. Sur le plan histologique, la tumeur peut être indifférenciée (carcinome embryonnaire), différenciée (tératome, tumeur du sac vitellin, choriocarcinome) ou constituée d'un mélange de composants séminomateux et non séminomateux."},"de":{"name":"Testikulärer Keimzelltumor, nicht-seminomatöser","synonyms":["Nicht-seminomatöser Keimzelltumor des Hodens"],"definition":"Eine Form des testikulären Keimzelltumors, die in der dritten Lebensdekade auftritt und sich in der Regel durch eine schmerzlose einseitige Gewebeverdichtung im Hodensack oder in einigen Fällen durch Gynäkomastie und/oder Rücken- und Flankenschmerzen bemerkbar macht. Der klinische Verlauf ist aggressiver als bei testikulären seminomatösen Keimzelltumoren, mit schneller Beteiligung von Blutgefäßen und einer schlechteren Prognose. Histologisch kann der Tumor entweder undifferenziert (embryonales Karzinom), differenziert (Teratom, Dottersacktumor, Choriokarzinom) sein oder aus einer Mischung von seminomatösen und nichtseminomatösen Komponenten bestehen."},"es":{"name":"Tumor testicular germinal no seminomatoso","synonyms":["Tumor germinal no disgerminomatoso de los testículos","Tumor germinal no seminomatoso de los testículos","Tumor testicular germinal no disgerminomatoso"],"definition":"Es un tipo de tumor testicular de células germinales que se presenta en la tercera década de la vida con una masa unilateral generalmente indolora en el escroto y, en algunos casos, con ginecomastia y/o dolor de espalda y en flanco. Se caracteriza por un curso clínico más agresivo que el de los tumores de células germinales seminomatosas testiculares, con una rápida afectación de los vasos sanguíneos y un pronóstico más desfavorable. Histológicamente, pueden ser tanto indiferenciados (carcinoma embrionario), como diferenciados (teratoma, tumor del saco vitelino, coriocarcinoma) o pueden constar de una mezcla de componentes seminomatosos y no seminomatosos."},"it":{"name":"Tumore testicolare non seminomatoso delle cellule germinali","synonyms":["Tumore non disgerminomatoso delle cellule germinali del testicolo","Tumore non seminomatoso delle cellule germinali del testicolo","Tumore testicolare non disgerminomatoso delle cellule germinali"],"definition":"Il tumore testicolare non seminomatoso delle cellule germinali comprende un gruppo di tumori testicolari delle cellule germinali (si veda questo termine) che esordiscono nella terza decade di vita (età media: 25 anni) con una massa monolaterale di solito indolore localizzata nello scroto oppure, in alcuni casi, con ginecomastia e/o dolore alla schiena e al fianco. Il decorso clinico è più aggressivo rispetto a quello dei tumori testicolari seminomatosi delle cellule germinali (si veda questo termine), con un rapido interessamento dei vasi sanguigni e una prognosi più sfavorevole. Dal punto di vista istologico, questi tumori sono indifferenziati (carcinoma embrionale), differenziati (teratoma, tumore del sacco vitellino, coriocarcinoma), oppure sono caratterizzati dall'associazione tra componenti seminomatose e non-seminomatose."},"nl":{"name":"Testiculaire niet-seminomateuze kiemceltumor","synonyms":["Niet-dysgerminomateuze kiemceltumor van de testis","Niet-seminomateuze kiemceltumor van de testis","Testiculaire niet-dysgerminomateuze kiemceltumor"],"definition":"Een vorm van testiculaire kiemceltumor die optreedt in het derde levensdecennium, met een meestal pijnloze unilaterale massa in scrotum of, in sommige gevallen, met gynaecomastie en/of pijn aan rug en flank. In vergelijking met testiculaire seminomateuze kiemceltumoren is het klinische verloop agressiever met snelle betrokkenheid van bloedvaten, en de prognose slechter. Histologisch kan de tumor niet-gedifferentieerd (embryonaal carcinoom) of gedifferentieerd (teratoom, dooierzaktumor, choriocarcinoom) zijn, of bestaan uit een mengeling van seminomateuze en niet-seminomateuze componenten."},"pt":{"name":"Tumor testicular de células germinativas não seminais"},"pl":{"name":"Nienasieniakowy zarodkowy nowotwór jądra","synonyms":["Guz jąder inny niż dysgerminoma"]},"cs":{"name":"Non-seminomový germinální nádor varlat","synonyms":["Non-dysgerminomatous germ cell tumor of testis","Testicular non seminomatous germ cell tumor","Testicular non-dysgerminomatous germ cell tumor"]},"uk":{"name":"Несеміноматозна герміногенна пухлина яєчка","synonyms":["Недисгерміноматозна герміногенна пухлина яєчка","Несеміноматозна герміногенна пухлина яєчка","Недисгерміноматозна герміногенна пухлина яєчка"],"definition":"A form of testicular germ cell tumor occurring in the third decade of life with a usually painless unilateral mass in the scrotum or, in some cases, with gynaecomastia and/or back and flack pain. The clinical course is more aggressive than testicular seminomatous germ cell tumors with rapid involvement of blood vessels and a poorer prognosis. Histologically, the tumour can be either undifferentiated (embryonal carcinoma), differentiated (teratoma, yolk sac tumor, choriocarcinoma), or can consist of a mixture of seminomatous and nonseminomatous components."},"zh":{"name":"非精原细胞睾丸生殖细胞瘤","synonyms":["睾丸非无性细胞性生殖细胞肿瘤"],"definition":"睾丸非精原细胞瘤描述了一组发生在二十至三十岁之间(平均年龄:25岁)的睾丸生殖细胞肿瘤(见本术语)，通常在阴囊可有无痛的单侧肿块，或在某些情况下有妇科疾病和/或背部及腰部疼痛，其特征是临床病程比睾丸精原细胞瘤(见本术语)更具侵袭性，血管受累快，预后较差。在组织学上，它们可以是未分化的(胚胎癌)、分化的(畸胎瘤、卵黄囊瘤、绒毛膜癌)，也可以由精原细胞性和非精原细胞性成分的混合物组成。"},"tr":{"name":"Testisin seminomatöz olmayan germ hücreli tümörü","synonyms":["Testiküler seminomatöz olmayan germ hücreli tümör","Testisin disgerminomatöz olmayan germ hücreli tümörü","Testiküler diserminomatöz olmayan germ hücreli tümör"]}},"xrefs":[{"source":"OMIM","code":"273300","qualifier":"NTBT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C62.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0006447","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2057625","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":363504,"name":"Germ cell tumor of testis","classifications":[185,202,233]}],"children":[],"genes":[{"symbol":"KITLG","name":"KIT ligand","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"19483682[PMID]_20228134[PMID]_23640991[PMID]","hgnc":"6343","ensembl":"ENSG00000049130","omim":"184745","loci":["12q21.32"]},{"symbol":"SPRY4","name":"sprouty RTK signaling antagonist 4","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"19483682[PMID]_23640991[PMID]","hgnc":"15533","ensembl":"ENSG00000187678","omim":"607984","loci":["5q31.3"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.21,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":33.53,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}