{"orpha":213610,"name":"Carcinosarcoma of the corpus uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Malignant mixed Müllerian tumor of the corpus uteri","Mixed Müllerian cancer of corpus uteri","Uterine carcinosarcoma"],"definition":"Carcinosarcoma of the corpus uteri is a rare, malignant, mixed epithelial and mesenchymal tumor of the uterine body composed of high-grade carcinomatous and sarcomatous elements. It may present with vaginal bleeding, abnormal vaginal discharge, abdominal pain and/or pelvic mass, with a polypoid tumor sometimes protruding through the cervical canal. Association with Tamoxifen therapy, long-term unopposed estrogen use and previous pelvic radiotherapy has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213610/","orphanet_url":"https://www.orpha.net/en/disease/detail/213610","names":{"fr":{"name":"Carcinosarcome du corps de l'utérus","synonyms":["Carcinosarcome utérin","Tumeur müllérienne mixte maligne du corps de l'utérus"],"definition":"Une tumeur maligne rare, mixte (épithéliale et mésenchymateuse) du corps de l'utérus, composée d'éléments sarcomateux et carcinomateux de haut grade. Elle peut se manifester par un saignement vaginal, une perte vaginale anormale, une douleur abdominale et/ou une masse pelvienne. Une tumeur polypoïde peut parfois dépasser du canal cervical. La tumeur peut être associée à l'utilisation de tamoxifène, à l'utilisation sur le long terme d'oestrogènes non compensés, et à une radiothérapie pelvienne antérieure."},"de":{"name":"Karzinosarkom des Corpus uteri","synonyms":["Müller-Mischtumor des Corpus uteri","Karzinosarkom, uterines","MMMT des Uterus"],"definition":"Das Karzinosarkom des Corpus uteri ist ein seltener, bösartiger, gemischter epithelialer und mesenchymaler Tumor des Gebärmutterkörpers, der aus hochgradig karzinomatösen und sarkomatösen Elementen besteht. Er kann sich mit vaginalen Blutungen, abnormalem vaginalen Ausfluss, Unterleibsschmerzen und/oder einer Beckenmasse präsentieren, wobei der polypoide Tumor manchmal durch den Zervikalkanal ragt. Es wurde über eine Assoziation mit einer Tamoxifen-Therapie, einer langfristigen Einnahme von unopposierten Östrogenen und einer vorangegangenen Strahlentherapie des Beckens berichtet."},"es":{"name":"Carcinosarcoma del cuerpo uterino","synonyms":["Carcinosarcoma uterino","Cáncer mülleriano mixto del cuerpo uterino","Tumor mülleriano mixto maligno del cuerpo uterino"],"definition":"El carcinosarcoma del cuerpo uterino es un tumor mixto mesenquimal-epitelial del cuerpo uterino, maligno y poco frecuente, compuesto por elementos carcinomatosos y sarcomatosos de alto grado. Puede presentarse con sangrado vaginal, flujo vaginal anómalo, dolor abdominal y/o masa pélvica, con un tumor polipoide que a veces protruye a través del canal cervical. Se ha notificado asociación con terapia con tamoxifeno, con el uso prolongado de estrógenos sin oposición y radioterapia pélvica previa."},"it":{"name":"Carcinoma del corpo dell'utero","definition":"Il carcinosarcoma del corpo dell'utero è un tumore maligno raro, misto epiteliale e mesenchimale, composto da elementi carcinomatosi e sarcomatosi con malignità di grado elevato. Esordisce con emorragie vaginali, perdite vaginali atipiche e dolore addominale, oppure sotto forma di massa pelvica, come tumore polipoide che talvolta protrude nel canale cervicale. È stato associato alla terapia con tamoxifene, con l'assunzione a lungo termine di estrogeni non controllati e con pregressi cicli di radioterapia pelvica."},"nl":{"name":"Carcinosarcoom van corpus uteri","synonyms":["Carcinosarcoom van baarmoederlichaam","Gemengde Mülleriaanse kanker van baarmoederlichaam","Gemengde Mülleriaanse kanker van corpus uteri","Maligne mengtumor van Müller van baarmoederlichaam","Maligne mengtumor van Müller van corpus uteri","Uterien carcinosarcoom"],"definition":"Carcinosarcoom van het corpus uteri is een zeldzame, maligne, gemengde epitheliale en mesenchymale tumor van het baarmoederlichaam die bestaat uit hooggradige carcinomateuze en sarcomateuze elementen. Het kan zich voordoen met vaginale bloeding, abnormale vaginale afscheiding, buikpijn en/of een massa in het bekken, met een polypoïde tumor die soms uitsteekt door het cervixkanaal. Er werd associatie gerapporteerd met Tamoxifen-therapie, langdurig ongehinderd gebruik van oestrogeen en voorafgaande radiotherapie van het bekken."},"pt":{"name":"Tumor misto mulleriano do corpo uterino"},"pl":{"name":"Mięsakorak trzonu macicy","synonyms":["Mięsakorak macicy","Rak mieszany Müllera trzonu macicy","Złośliwy rak mieszany Müllera trzonu macicy"]},"cs":{"name":"Zhoubný smíšený mülleriánský nádor těla děložního","synonyms":["Smíšená mülleriánská rakovina těla děložního"]},"uk":{"name":"Карциносаркома тіла матки","synonyms":["Злоякісна змішана мюллерова пухлина тіла матки","Змішаний мюллерівський рак тіла матки","Карциносаркома матки"],"definition":"Carcinosarcoma of the corpus uteri is a rare, malignant, mixed epithelial and mesenchymal tumor of the uterine body composed of high-grade carcinomatous and sarcomatous elements. It may present with vaginal bleeding, abnormal vaginal discharge, abdominal pain and/or pelvic mass, with a polypoid tumor sometimes protruding through the cervical canal. Association with Tamoxifen therapy, long-term unopposed estrogen use and previous pelvic radiotherapy has been reported."},"zh":{"name":"子宫体癌肉瘤","synonyms":["子宫体恶性混合型苗勒管肿瘤"],"definition":"子宫体癌肉瘤是一种罕见的、恶性的、混合上皮和间充质的子宫体肿瘤，由高级癌变和肉瘤成分组成。本病临床表现为阴道出血、异常阴道分泌物、腹痛和/或盆腔肿块，伴有可能突出于宫颈管的息肉样肿瘤。曾报道与他莫昔芬治疗，长期高雌激素暴露以及既往盆腔放疗相关。"},"tr":{"name":"Korpus uteri karsinosarkomu","synonyms":["Uterin karsinosarkom","Korpus uteri'nin karma Müllerian kanseri","Korpus uteri'nin habis karma Müllerian tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C54.9","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C76.43","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1711927798"},{"source":"MONDO","code":"0016259","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1704376","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12335","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213589,"name":"Malignant mixed epithelial and mesenchymal tumor of corpus uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}