{"orpha":424943,"name":"Adenocarcinoma of the liver and intrahepatic biliary tract","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adenocarcinoma of the liver and IBT"],"definition":"A rare hepatic and biliary tract tumor characterized by a growth pattern ressembling that found in hepatocellular carcinomas and cholangiocarcinomas but presenting atypical histological and immunohistochemical features (such as trabecular, organoid, microcystic and/or blastemal-like architecture and inhibin A, cytokeratin 7 and/or cytokeratin 19 positivity) that do not allow a formal diagnosis of the more common aforementioned liver cancers. Patients may present abdominal distension and pain, a palpable abdominal mass and elevated liver enzymes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424943/","orphanet_url":"https://www.orpha.net/en/disease/detail/424943","names":{"fr":{"name":"Adénocarcinome du foie et des voies biliaires intrahépatiques","synonyms":["Adénocarcinome du foie et des VBIH"],"definition":"Tumeur rare du foie et des voies biliaires caractérisée par un développement ressemblant à celui des carcinomes hépatocellulaires et des cholangiocarcinomes, mais dont les caractéristiques histologiques et immunohistochimiques, atypiques (architecture trabéculaire, organoïde, microkystique et/ou similaire à celle du blastème, et positivité à l'inhibine A, la cytokératine 7 et/ou la cytokératine 19), ne permettent pas d'établir un diagnostic officiel des cancers hépatiques susmentionnés, plus courants. Les patients peuvent présenter une distension et des douleurs abdominales, ainsi qu'une masse abdominale palpable à l'examen et un taux élevé d'enzymes hépatiques."},"de":{"name":"Adenokarzinom der Leber und intrahepatischen Gallengänge","definition":"Ein seltener Tumor der Leber und der Gallenwege, der durch ein Wachstumsmuster gekennzeichnet ist, das dem hepatozellulärer Karzinome und Cholangiokarzinome ähnelt, aber atypische histologische und immunhistochemische Merkmale aufweist (z.B. trabekuläre, organoide, mikrozystische und/oder blastemale Architektur und positiver Nachweis von Inhibin A, Cytokeratin 7 und/oder Cytokeratin 19), die eine formale Diagnose der häufigeren vorgenannten Leberkrebsarten nicht zulassen. Die Patienten können einen aufgetriebenen Bauch, Schmerzen, eine tastbare abdominale Masse und erhöhte Leberenzyme aufweisen."},"es":{"name":"Adenocarcinoma de hígado y vías biliares intrahepáticas","synonyms":["Adenocarcinoma de hígado y VBI"],"definition":"Es un tumor hepático y de las vías biliares poco frecuente caracterizado por un patrón de crecimiento similar al encontrado en los carcinomas hepatocelulares y en los colangiocarcinomas pero que presenta hallazgos histológicos e inmunohistoquímicos atípicos (tales como arquitectura trabecular, organoide, microquística y/o de tipo blastema y expresión de inhibina A, citoqueratina 7 y/o citoqueratina 19) que impiden el diagnóstico formal de los tumores hepáticos más comunes mencionados con anterioridad. Los afectados pueden presentar distensión y dolor abdominal, una masa abdominal palpable y elevación de las enzimas hepáticas."},"it":{"name":"Adenocarcinoma del fegato e delle vie biliari intraepatiche","synonyms":["Adenocarcinoma del fegato e delle VBI"],"definition":"È una neoplasia molto rara del fegato e delle vie biliari, che presenta una crescita simile a quella dei carcinomi epatocellulari e dei colangiocarcinomi, ma con caratteristiche istologiche ed immunoistochimiche atipiche (architettura trabecolare, organoide, microcistica e/o simil-blastemale, positiva all'inibina A, alla citocheratina 7 e/o alla citocheratina 19) che non permettono di classificarla formalmente tra i più comuni tumori epatici sopra menzionati. I pazienti possono presentare una distensione e un dolore addominale, una massa addominale palpabile e un aumento degli enzimi epatici."},"nl":{"name":"Adenocarcinoom van lever en intrahepatische galwegen","synonyms":["Adenocarcinoom van lever en IBT"],"definition":"Een zeldzame tumor van lever en galwegen, gekenmerkt door een groeipatroon gelijkaardig aan dat van hepatocellulaire carcinomen en cholangiocarcinomen, maar met atypische histologische en immunohistochemische kenmerken (zoals trabeculaire, organoïde, microcystische en/of blasteem-achtige architectuur en expressie van inhibine A, cytokeratine 7 en/of cytokeratine 19), die niet toelaten een formele diagnose van deze meer gangbare leverkankers te stellen. Patiënten vertonen mogelijk abdominale distensie en pijn, een tastbare abdominale massa en verhoogde gehaltes van leverenzymen."},"pt":{"name":"Adenocarcinoma do fígado e do trato biliar intra-hepático","synonyms":["Adenocarcinoma do fígado e do TBIH","Adenocarcinoma hepático e do trato biliar intra-hepático"]},"pl":{"name":"Gruczolakorak wątroby i wewnątrzwątrobowego przewodu żółciowego","synonyms":["Gruczolakorak wątroby i IBT"],"definition":"Rzadki nowotwór wątroby i dróg żółciowych charakteryzujący się wzorcem wzrostu obserwowanym w rakach wątrobowokomórkowych i rakach dróg żółciowych, ale o nietypowych cechach histologicznych i immunohistochemicznych (architektura przypominającą blastemę i/lub beleczkowa, organoidalna, mikropęcherzykowa oraz dodatni wynik badania w kierunku inhibiny A, cytokeratyny 7 i/lub cytokeratyny), które nie pozwalają na formalne rozpoznanie częściej występujących nowotworów, które wymieniono powyżej. Może wystąpić wzdęcie i ból brzucha, wyczuwalny guz w jamie brzusznej i podwyższone enzymy wątrobowe."},"cs":{"name":"Adenoarcinom jater a intrahepatálních žlučových cest","synonyms":["Adenokarcinom jater a IBT"]},"uk":{"name":"Аденокарцинома печінки та внутрішньопечінкових жовчних шляхів","synonyms":["Аденокарцинома печінки та IBT"],"definition":"A rare hepatic and biliary tract tumor characterized by a growth pattern ressembling that found in hepatocellular carcinomas and cholangiocarcinomas but presenting atypical histological and immunohistochemical features (such as trabecular, organoid, microcystic and/or blastemal-like architecture and inhibin A, cytokeratin 7 and/or cytokeratin 19 positivity) that do not allow a formal diagnosis of the more common aforementioned liver cancers. Patients may present abdominal distension and pain, a palpable abdominal mass and elevated liver enzymes."},"zh":{"name":"肝和肝内胆道腺癌","synonyms":["肝腺癌和IBT"],"definition":"一种非常罕见的肝胆道肿瘤，其发展方式与肝细胞癌和胆管癌相似，但具有非典型的组织学和免疫组织化学特征（如小梁状、类器官、微囊和/或胚芽样结构，以及抑制素A、细胞角蛋白7和/或细胞角蛋白19阳性），不能采用同前述肝癌相同诊断方法。患者可能出现腹胀和疼痛，可触及腹部肿块和肝酶升高。"},"tr":{"name":"Karaciğer adenokarsinomu ve intrahepatik safra yolları","synonyms":["Karaciğer adenokarsinomu ve IBT"]}},"xrefs":[{"source":"ICD-10","code":"C22.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018532","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5192429","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":424936,"name":"Carcinoma of liver and intrahepatic 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 / 1 000 000","value":0.412,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.715,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.201,"area":"Belgium","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.219,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.659,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.471,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.309,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.262,"area":"France","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.582,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.256,"area":"Iceland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.642,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.426,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.267,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.481,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.365,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.154,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.471,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.377,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.753,"area":"Croatia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.157,"area":"Ireland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.206,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.467,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.118,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}