{"orpha":213787,"name":"Carcinosarcoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Cervical carcinosarcoma","Cervical malignant Müllerian mixed tumor","Malignant Müllerian mixed tumor of the cervix uteri"],"definition":"Carcinosarcoma of the cervix uteri is a rare, malignant, mixed epithelial and mesenchymal tumor, located in the cervix uteri, composed of an admixture of carcinomatous and sarcomatous elements. It usually presents with abnormal vaginal bleeding and a round, well-defined, grey to yellowish-white, pedunculated polypoid mass protruding through the cervical canal. Association with HPV infection (especially serotype 16) has been frequently reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213787/","orphanet_url":"https://www.orpha.net/en/disease/detail/213787","names":{"fr":{"name":"Carcinosarcome du col de l'utérus","synonyms":["Carcinosarcome cervicale","Carcinosarcome du col utérin","Tumeur müllérienne mixte maligne cervicale","Tumeur müllérienne mixte maligne du col de l'utérus"],"definition":"Une tumeur rare, maligne, mixte, épithéliale et mésenchymateuse, située au niveau du col de l'utérus et composée d'un mélange d'éléments carcinomateux et sarcomateux. Elle se manifeste habituellement par un saignement vaginal anormal et par une masse polypoïde pédonculée arrondie, bien définie, de coloration grise à blanc-jaunâtre, qui dépasse à travers le canal cervical. La tumeur est fréquemment associée à une infection par l'HPV (surtout le sérotype 16)."},"de":{"name":"Karzinosarkom der Cervix uteri","synonyms":["Karzinosarkom, zervikales","MMMT der Cervix uteri","Maligner Müller-Mischtumor der Cervix uteri","Müller-Mischtumor, zervikaler"],"definition":"Das Karzinosarkom der Cervix uteri ist ein seltener, maligner, gemischter epithelialer und mesenchymaler Tumor, der im Gebärmutterhals lokalisiert ist und aus einer Mischung von karzinomatösen und sarkomatösen Elementen besteht. Er präsentiert sich meist mit abnormalen vaginalen Blutungen und einer runden, gut definierten, grauen bis gelblich-weißen, gestielten polypoiden Masse, die durch den Zervikalkanal ragt. Eine Assoziation mit einer HPV-Infektion (insbesondere Serotyp 16) ist häufig berichtet worden."},"es":{"name":"Carcinosarcoma del cuello uterino","synonyms":["Carcinosarcoma cervical","Tumor mülleriano mixto maligno cervical","Tumor mülleriano mixto maligno del cuello uterino"],"definition":"El carcinosarcoma del cuello uterino es un tumor mixto epitelial y mesenquimal, maligno y poco frecuente, localizado en el cuello uterino, compuesto por una mezcla de elementos carcinomatosos y sarcomatosos. Por lo general, presenta sangrado vaginal anómalo y una masa polipoidea pediculada, redondeada, bien definida, de color gris a blanco amarillento, que protruye a través del canal cervical. Con frecuencia se asocia con infección por VPH (especialmente el serotipo 16)."},"it":{"name":"Carcinosarcoma della cervice uterina","definition":"Il carcinosarcoma della cervice uterina è un tumore maligno raro, misto epiteliale e mesenchimale, caratterizzato dall'associazione di elementi carcinomatosi e sarcomatosi. Di solito esordisce con emorragie vaginali atipiche e con una massa polipoide peduncolata, rotonda, ben definita, di colore grigio o giallastro-bianco, che protrude nel canale cervicale. Spesso si associa all'infezione da HPV (soprattutto il sierotipo 16)."},"nl":{"name":"Carcinosarcoom van cervix uteri","synonyms":["Carcinosarcoom van baarmoederhals","Cervicaal carcinosarcoom","Cervicale maligne mengtumor van Müller","Maligne mengtumor van Müller van baarmoederhals","Maligne mengtumor van Müller van cervix uteri"],"definition":"Carcinosarcoom van de cervix uteri is een zeldzame, maligne, gemengde epitheliale en mesenchymale tumor die is gelokaliseerd in de baarmoederhals en bestaat uit een mengeling van carcinomateuze en sarcomateuze elementen. Meestal manifesteert het zich met abnormale vaginale bloeding en een ronde, goed gedefinieerde, grijze tot geel-witte, gesteelde polypoïde massa die uitsteekt door het cervixkanaal. Associatie met HPV-infectie (voornamelijk serotype 16) werd reeds frequent gerapporteerd."},"pt":{"name":"Tumor maligno misto mulleriano do colo do útero"},"pl":{"name":"Mięsakorak szyjki macicy","synonyms":["Rak mieszany Müllera szyjki macicy","Złośliwy rak mieszany Müllera szyjki macicy"]},"cs":{"name":"Maligní smíšený mülleriánský nádor hrdla děložního","synonyms":["Cervikální maligní smíšený mulleriánský nádor"]},"uk":{"name":"Карциносаркома шийки матки","synonyms":["Карциносаркома шийки матки","Злоякісна мюллерівська змішана пухлина шийки матки","Злоякісна мюллерівська змішана пухлина шийки матки"],"definition":"Carcinosarcoma of the cervix uteri is a rare, malignant, mixed epithelial and mesenchymal tumor, located in the cervix uteri, composed of an admixture of carcinomatous and sarcomatous elements. It usually presents with abnormal vaginal bleeding and a round, well-defined, grey to yellowish-white, pedunculated polypoid mass protruding through the cervical canal. Association with HPV infection (especially serotype 16) has been frequently reported."},"zh":{"name":"子宫颈癌肉瘤","synonyms":["子宫颈恶性苗勒管混合性肿瘤"],"definition":"子宫颈癌肉瘤是一种罕见的恶性混合上皮间质瘤，位于子宫颈，由癌细胞和肉瘤成分混合而成。它通常表现为异常阴道出血和一个圆形、界限清楚、灰色至黄白色、有蒂息肉样的肿块，突出于宫颈管。以往常报道与HPV感染（尤其是血清型16）相关。"},"tr":{"name":"Serviks uterinin karsinosarkomu","synonyms":["Servikal karsinosarkom","Servikal habis Müllerian karma tümör","Serviks uteri habis Müllerian karma tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C53","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C77.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2030254088"},{"source":"MONDO","code":"0002877","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1332917","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213782,"name":"Malignant mixed epithelial and mesenchymal tumor of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_26023676[PMID]"}],"phenotypes":null}