{"orpha":99978,"name":"Perihilar cholangiocarcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Hilar CCA","Hilar cholangiocarcinoma","Klatskin tumor"],"definition":"Klatskin tumor is an extra-hepatic cholangiocarcinoma (CCA) arising in the junction of the main right or left hepatic ducts to form the common hepatic duct.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99978/","orphanet_url":"https://www.orpha.net/en/disease/detail/99978","names":{"fr":{"name":"Cholangiocarcinome périhilaire","synonyms":["CCA hilaire","Cholangiocarcinome hilaire","Tumeur de Klatskin"],"definition":"La tumeur de Klatskin est un cholangiocarcinome (CCA) extra-hépatique survenant à la jonction des canaux hépatiques droit et gauche formant le canal hépatique commun."},"de":{"name":"Cholangiokarzinom, perihiläres","synonyms":["Hiläres Gallengangkarzinom","Klatskin-Tumor","Perihiläres CCA"],"definition":"Der Klatskin-Tumor ist ein extra-hepatisches Cholangiokarzinom (CCA, siehe diesen Begriff), das an der Verbindungsstelle vom großen rechten und großem linken Lebergang, am Beginn des gemeinsamen Leberganges auftritt."},"es":{"name":"Colangiocarcinoma perihiliar","synonyms":["CCA hiliar","Colangiocarcinoma hiliar","Tumor de Klatskin"],"definition":"El tumor de Klatskin es un colangiocarcinoma (CCA, consulte este término) extra-hepático que aparece en la unión de los conductos hepáticos principales derecho o izquierdo para formar el conducto hepático común."},"it":{"name":"Tumore di Klatskin","synonyms":["Colangiocarcinoma ilare","CCA ilare"],"definition":"Il tumore di Klatskin è un colangiosarcoma extraepatico (CCA; si veda questo termine) che insorge nella giunzione dei principali dotti epatici, destro o sinistro, che formano il dotto epatico comune."},"nl":{"name":"Perihilair cholangiocarcinoom","synonyms":["Hilair CCA","Hilair cholangiocarcinoom","Klatskin-tumor"],"definition":"Klatskin-tumor is een extrahepatisch cholangiocarcinoom (CCA, zie deze term) dat zich ontwikkelt op de plaats waar linker of rechter ductus hepaticus samenkomen om de gemeenschappelijke ductus hepaticus te vormen."},"pt":{"name":"Colangiocarcinoma peri-hilar","synonyms":["CCA hilar","Colangiocarcinoma hilar","Tumor de Klatskin"]},"pl":{"name":"Guz Klatskina","synonyms":["Hilar CCA","Hilar cholangiocarcinoma"]},"cs":{"name":"Klatskinův nádor","synonyms":["Hilový CCA","Hilový cholangiokarcinom"]},"uk":{"name":"Пухлина Клацкіна","synonyms":["Хилярна CCA","Хилярна холангіокарцинома"],"definition":"Klatskin tumor is an extra-hepatic cholangiocarcinoma (CCA, see this term) arising in the junction of the main right or left hepatic ducts to form the common hepatic duct."},"zh":{"name":"克拉斯金肿瘤","synonyms":["肝门部胆管癌"],"definition":"肝门部胆管癌是一种肝外胆管癌(CCA，见该词条)，发生在主要的右或左肝管形成总肝管的交界处。"},"tr":{"name":"Klatskin tümörü","synonyms":["Hilar CCA","Hiler kolanjiyokarsinom"]}},"xrefs":[{"source":"ICD-10","code":"C24.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C18.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1571104786"},{"source":"MONDO","code":"0003345","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018285","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206702","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10175","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":56044,"name":"Carcinoma of gallbladder and extrahepatic biliary tract","classifications":[183,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.5,"area":"United States","status":"Not yet validated","source":"19638920[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0030153","label":"Cholangiocarcinoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012334","label":"Extrahepatic cholestasis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004936","label":"Venous thrombosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}