{"orpha":252212,"name":"Malignant triton tumor","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["MPNST with rhabdomyosarcomatous differentiation","MTT","Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differenciation"],"definition":"Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized histopathologically by focal rhabdomyoblastic differentiation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-252212/","orphanet_url":"https://www.orpha.net/en/disease/detail/252212","names":{"fr":{"name":"Tumeur triton maligne","synonyms":["MPNST avec différenciation rhabdomyosarcomateuse","TTM","Tumeur maligne des gaines nerveuses avec différenciation rhabdomyosarcomateuse"],"definition":"Sous-type agressif rare de tumeur maligne des gaines nerveuses périphériques caractérisé, sur le plan histopathologique, par une différenciation rhabdomyoblastique focale."},"de":{"name":"Triton-Tumor, maligner","synonyms":["MPNST mit rhabdomyosarkomatöser Differenzierung","MTT","Nervenscheidentumor, maligner peripherer, mit rhabdomyosarkomatöser Differenzierung"]},"es":{"name":"Tumor tritón maligno","synonyms":["MPNST con diferenciación rabdomiosarcomatosa","MTT","Tumor maligno de vaina nerviosa periférica con diferenciación rabdomiosarcomatosa"],"definition":"El tumor tritón maligno (MTT) es un subtipo agresivo raro de tumor maligno de la vaina nerviosa periférica (MPNST; consulte este término) caracterizado histopatológicamente por diferenciación rabdomioblástica focal."},"it":{"name":"Tumore maligno del tritone","synonyms":["MPNST con differenziazione rabdomiosarcomatosa","MTT","Tumore maligno delle guaine nervose con differenziazione rabdomiosarcomatosa"],"definition":"Il tumore maligno del tritone (MTT) è un raro sottotipo aggressivo del tumore maligno della guaina dei nervi periferici (MPNST; si veda questo termine) caratterizzato, dal punto di vista istopatologico, dalla differenziazione rabdomioblastica focale."},"nl":{"name":"Maligne tritontumor","synonyms":["MTT","Maligne tumor van perifere zenuwschede met rhabdomyosarcomateuze differentiatie","Kwaadaardige tritontumor","MPNST met rhabdomyosarcomateuze differentiatie"],"definition":"Maligne tritontumor (MTT) is een zeldzaam, agressief subtype van maligne perifere zenuwschedetumor (MPNST; zie deze term) dat histopathologisch wordt gekarakteriseerd door focale rhabdomyoblastische differentiatie."},"pt":{"name":"Tumor tritão maligno","synonyms":["MPNST com diferenciação rabdomiossarcomatosa","MTT","Tumor maligno da bainha dos nervos periféricos com diferenciação rabdomiossarcomatosa"]},"pl":{"name":"Złośliwy guz Trytona","synonyms":["MPNST z różnicowaniem w kierunku mięśniakomięsaka prążkowanokomórkowego","MTT","Złośliwy nowotwór osłonek nerwowych z różnicowaniem w kierunku mięśniakomięsaka prążkowanokomórkowego"],"definition":"Złośliwy guz Trytona (MTT) jest rzadkim agresywnym podtypem złośliwego nowotworu osłonek nerwów obwodowych (MPNST); histopatologicznie charakteryzuje się obszarami różnicowania w kierunku mięsaka z mięśni poprzecznie prążkowanych."},"cs":{"name":"Maligní Tritonův tumor","synonyms":["MPNST s rhabdomyosarkomatózní diferenciací","MTT","Zhoubný poševní nádor periferních nervů s rhabdomyosarkomatózní diferenciací"]},"uk":{"name":"Злоякісна пухлина тритона","synonyms":["Злоякісна пухлина оболонки периферичного нерва з рабдоміосаркоматозним диференціюванням","МПНСТ з рабдоміосаркоматозною диференціацією","MTT"],"definition":"Malignant triton tumor (MTT) is a rare aggressive subtype of malignant peripheral nerve sheath tumor (MPNST; see this term) characterized histopathologically by focal rhabdomyoblastic differentiation."},"zh":{"name":"恶性蝾螈瘤","synonyms":["恶性神经鞘瘤伴横纹肌肉瘤分化"],"definition":"恶性Triton瘤(MTT)是恶性周围神经鞘瘤(MPNST)的一种罕见的侵袭性亚型，其组织病理学特征为局灶性横纹肌母细胞分化。"},"tr":{"name":"Habis triton tümörü","synonyms":["MTT","Rabdomyosarkomatöz farklılaşma ile seyreden MPNST","Rabdomyosarkomatöz farklılaşma ile seyreden habis periferik sinir kılıfı tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C47.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"XH2VV8","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"867726878"},{"source":"MONDO","code":"0016757","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334616","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":3148,"name":"Malignant peripheral nerve sheath tumor","classifications":[181,202]}],"children":[],"genes":[{"symbol":"HTRA1","name":"HtrA serine peptidase 1","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"27723760[PMID]","hgnc":"9476","ensembl":"ENSG00000166033","omim":"602194","loci":["10q26.13"]},{"symbol":"SH3PXD2A","name":"SH3 and PX domains 2A","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"27723760[PMID]","hgnc":"23664","ensembl":"ENSG00000107957","omim":"619455","loci":["10q24.33"]}],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":170.0,"area":"Worldwide","status":"Validated","source":"24474920[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_24474920[PMID]"}],"phenotypes":null}