{"orpha":443909,"name":"Hereditary nonpolyposis colon cancer","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Familial nonpolyposis colon cancer","Familial nonpolyposis colorectal cancer","HNPCC","Hereditary nonpolyposis colorectal cancer"],"definition":"A cancer-predisposing condition characterized by the development of colorectal cancer not associated with colorectal polyposis, endometrial cancer, and various other cancers (such as malignant epithelial tumor of ovary, gastric, biliary tract, small bowel, and urinary tract cancer) that are frequently diagnosed at an early age.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-443909/","orphanet_url":"https://www.orpha.net/en/disease/detail/443909","names":{"fr":{"name":"Cancer du côlon héréditaire non polyposique","synonyms":["Cancer colorectal familial sans polypose","Cancer colorectal héréditaire sans polypose","Cancer du côlon familial non polyposique","HNPCC"],"definition":"Maladie de prédisposition aux cancers caractérisée par le développement d'un cancer colorectal non associé à une polypose colorectale, d'un cancer de l'endomètre et de divers autres cancers (tels que la tumeur épithéliale maligne de l'ovaire, le cancer de l'estomac, des voies biliaires, de l'intestin grêle et des voies urinaires) qui sont fréquemment diagnostiqués à un jeune âge."},"de":{"name":"Kolonkarzinom, hereditäres nicht-polypöses","synonyms":["HNPCC","Karzinom, kolorektales nicht-polypöses, familiäre Form","Kolonkarzinom, familiäres nicht-polypöses"],"definition":"Das hereditäre nicht-polypöse Kolonkarzinom (HNPCC) ist eine krebsprädisponierende Erkrankung, die durch die Entwicklung von Darmkrebs gekennzeichnet ist. Sie ist nicht mit dem Auftreten von kolorektalen Polypen, Endometriumkrebs oder verschiedenen anderen Krebsarten (wie maligner epithelialer Tumor des Eierstocks, Magen-, Gallen-, Dünndarm- und Harnwegskrebs) assoziiert, die häufig bereits im frühen Alter diagnostiziert werden."},"es":{"name":"Cáncer de colon no polipósico hereditario","synonyms":["Cáncer colorrectal no polipósico familiar","Cáncer colorrectal no polipósico hereditario","Cáncer de colon no polipósico familiar","HNPCC"],"definition":"Es una condición de predisposición al cáncer caracterizada por el desarrollo de cáncer colorrectal no asociado a poliposis colorrectal, cáncer endometrial y diversos tipos de cáncer (tales como tumor ovárico epitelial maligno, cáncer gástrico, de las vías biliares, del intestino delgado y del tracto urinario) que son diagnosticados, con frecuencia, a una edad temprana."},"it":{"name":"Carcinoma non poliposico ereditario del colon","synonyms":["Carcinoma colorettale non poliposico ereditario","Carcinoma colorettale non poliposico familiare","Carcinoma del colon non poliposico familiare","HNPCC"],"definition":"Si tratta di una condizione di suscettibilità ai tumori, caratterizzata dallo sviluppo di un tumore colorettale non associato alla poliposi colorettale, di un tumore dell'endometrio e di altre neoplasie (ad es. il tumore epiteliale maligno dell'ovaio, il tumore gastrico, delle vie biliari, dell'intestino tenue e delle vie urinarie), che spesso vengono diagnosticati in giovane età."},"nl":{"name":"Hereditaire non-polyposis colonkanker","synonyms":["Erfelijke non-polyposis colorectale kanker","Erfelijke non-polyposis dikkedarmkanker","Familiale non-polyposis colonkanker","Familiale non-polyposis colorectale kanker","HNPCC"],"definition":"Een kankerpredispositie gekarakteriseerd door de ontwikkeling van colorectale kanker (CRC) niet geassocieerd met colorectale polyposis, kanker van endometrium, en verscheidene andere kankers (zoals kwaadaardige epitheeltumoren van ovarium, maag, galsysteem, dunne darm, en urinewegen) die veelal op een vroege leeftijd gediagnosticeerd worden."},"pt":{"name":"Cancro de cólon hereditário sem polipose"},"pl":{"name":"Dziedziczny niepolipowaty rak jelita grubego","synonyms":["HNPCC","Rodzinnny niepolipowaty rak jelita grubego","Rodzinnny niepolipowaty rak jelita grubego"]},"cs":{"name":"Hereditární nepolypózní karcinom kolorekta","synonyms":["HNPCC","Hereditární nepolypózní kolorektální karcinom","Familiární nepolypózní nádor střeva","Hereditární nepolypózní nádor střeva"]},"uk":{"name":"Спадковий неполіпозний рак товстої кишки","synonyms":["Сімейний неполіпозний рак товстої кишки","Сімейний неполіпозний колоректальний рак","Спадковий неполіпозний колоректальний рак","HNPCC"],"definition":"A cancer-predisposing condition characterized by the development of colorectal cancer not associated with colorectal polyposis, endometrial cancer, and various other cancers (such as malignant epithelial tumor of ovary, gastric, biliary tract, small bowel, and urinary tract cancer) that are frequently diagnosed at an early age."},"zh":{"name":"遗传性非息肉病性结肠癌","synonyms":["遗传性非息肉病性结直肠癌"],"definition":"一种癌症倾向，特征为可发展为大肠癌且不合并大肠息肉病，子宫内膜癌和各种其他癌症（例如卵巢，胃，胆道，小肠和尿道癌的恶性上皮肿瘤）。上述肿瘤在早期即可被诊断。"},"tr":{"name":"Kalıtsal polipozis dışı kolon kanseri","synonyms":["HNPCC","Ailesel polipozis dışı kolon kanseri","Ailesel polipozis dışı kolorektal kanser","Kalıtsal polipozis dışı kolorektal kanser"]}},"xrefs":[{"source":"MONDO","code":"0018630","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18630","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333990","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8533","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":271835,"name":"Genetic digestive tract tumor","classifications":[156]},{"orpha":425003,"name":"Inherited digestive cancer-predisposing syndrome","classifications":[152,156,202]},{"orpha":589746,"name":"Inherited gynecological cancer-predisposing syndrome","classifications":[156,202]}],"children":[{"orpha":440437,"name":"Familial colorectal cancer Type X","type":"Disease"},{"orpha":144,"name":"Lynch syndrome","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}