{"orpha":424991,"name":"Adenocarcinoma of the gallbladder and extrahepatic biliary tract","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adenocarcinoma of the gallbladder and EBT"],"definition":"A rare epithelial carcinoma, arising either in the gallbladder itself or from the epithelium lining the extrahepatic biliary tree, cystic duct and/or peribiliary gland, characterized by nonspecific symptoms, such as abdominal pain, jaundice and vomiting and sometimes mimicking benign biliary diseases. Chronic biliary epithelial inflammation (e.g. primary sclerosing cholangitis, cholelithiasis, choledocholithiasis, liver fluke infestation) is a major risk factor.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424991/","orphanet_url":"https://www.orpha.net/en/disease/detail/424991","names":{"fr":{"name":"Adénocarcinome de la vésicule biliaire et des voies biliaires extrahépatiques","synonyms":["Adénocarcinome de la vésicule biliaire et des VBEH"],"definition":"Un carcinome épithélial rare apparaissant soit au niveau de la vésicule biliaire ou au niveau de l'épithélium bordant l'arbre biliaire extra-hépatique, le canal cystique et/ou la glande péri-biliaire. Il se caractérise par des symptômes non spécifiques telles qu'une douleur abdominale, un ictère et des vomissements, et mime parfois des maladies biliaires bénignes. L'inflammation épithéliale biliaire chronique (comme une cholangite sclérosante primitive, une cholélithiase, une cholédocholithiase, une infestation pulmonaire par des douves) constitue un facteur de risque majeur."},"de":{"name":"Adenokarzinom der Gallenblase und extrahepatischen Gallengänge","synonyms":["Adenokarzinom der Gallenblase und EBT"],"definition":"Ein seltenes Epithelkarzinom, das entweder von der Gallenblase selbst ausgeht, oder von dem Epithel, das den extrahepatischen Gallenbaum, den Ductus cysticus und/oder die peribiliäre Drüse auskleidet. Es manifestiert sich durch unspezifische Symptome wie Bauchschmerzen, Gelbsucht und Erbrechen und imitiert manchmal gutartige Gallenwegerkrankungen. Die chronische Entzündung des biliären Epithels (z. B. bei primär sklerosierender Cholangitis, Cholelithiasis, Choledocholithiasis, Leberegelbefall) stellt ein Hauptrisikofaktor dar."},"es":{"name":"Adenocarcinoma de la vesícula biliar y de las vías biliares extrahepáticas","synonyms":["Adenocarcinoma de vesícula y VBE"],"definition":"Es un carcinoma epitelial poco frecuente que se origina en la vesícula biliar o en el epitelio que recubre el árbol biliar extrahepático, el conducto cístico y/o la glándula peribiliar. Está caracterizado por síntomas inespecíficos, tales como dolor abdominal, ictericia y vómitos, simulando en ocasiones enfermedades biliares benignas. La inflamación crónica del epitelio biliar (p. ej., colangitis esclerosante primaria, colelitiasis, coledocolitiasis, infestación por fasciola hepática) es un factor de riesgo importante."},"it":{"name":"Adenocarcinoma della cistifellea e delle vie biliari extraepatiche","synonyms":["Adenocarcinoma della cistifellea e delle VBE"],"definition":"L'adenocarcinoma della cistifellea e delle vie biliari extraepatiche è un carcinoma epiteliale raro, che origina dalla cistifellea oppure dall'epitelio che riveste l'albero biliare extraepatico, il dotto cistico e/o la ghiandola peribiliare. È caratterizzato da sintomi aspecifici, come il dolore addominale, l'ittero e il vomito, che talvolta ricordano quelli delle malattie biliari benigne. L'infiammazione cronica dell'epitelio biliare (colangite sclerosante primitiva, colelitiasi, coledocolitiasi, infestazione da distoma epatico) rappresenta un importante fattore di rischio."},"nl":{"name":"Adenocarcinoom van galblaas en extrahepatische galwegen","synonyms":["Adenocarcinoom van galblaas en EBT"],"definition":"Een zeldzaam, epitheliaal carcinoom dat ontstaat in de galblaas zelf of uit het epitheel van de extrahepatische galboom, de galblaasafvoergang en/of peribiliaire klieren, en dat gekarakteriseerd wordt door niet-specifieke symptomen zoals buikpijn, geelzucht en braken, en dat soms lijkt op goedaardige galaandoeningen. Chronisch inflammatie van galwegepitheel (e.g. primaire scleroserende cholangitis, cholelithiasis, choledocholithiasis, leverbotbesmetting) is een belangrijke risicofactor."},"pt":{"name":"Adenocarcinoma da vesícula biliar e do trato biliar extra-hepático","synonyms":["Adenocarcinoma da vesícula biliar e do TBEH"]},"pl":{"name":"Gruczolakorak pęcherzyka żółciowego i zewnątrzwątrobowego przewodu żółciowego","synonyms":["Gruczolakorak pęcherzyka żółciowego i EBT"]},"cs":{"name":"Adenokarcinom žlučníku a extrahepatálních žlučových cest","synonyms":["Adenokarcinom žlučníku a EBT"]},"uk":{"name":"Аденокарцинома жовчного міхура та позапечінкових жовчовивідних шляхів","synonyms":["Аденокарцинома жовчного міхура та EBT"],"definition":"A rare epithelial carcinoma, arising either in the gallbladder itself or from the epithelium lining the extrahepatic biliary tree, cystic duct and/or peribiliary gland, characterized by nonspecific symptoms, such as abdominal pain, jaundice and vomiting and sometimes mimicking benign biliary diseases. Chronic biliary epithelial inflammation (e.g. primary sclerosing cholangitis, cholelithiasis, choledocholithiasis, liver fluke infestation) is a major risk factor."},"zh":{"name":"胆囊和肝外胆道腺癌","synonyms":["胆囊腺癌和EBT"],"definition":"胆囊癌一种罕见的上皮性癌，起源于胆囊本身或肝外胆管、胆囊管和/或胆周腺的上皮，其临床表型为非特异性症状，如腹痛、黄疸和呕吐，有类似良性胆道疾病。慢性胆道上皮炎症（如原发性硬化性胆管炎、胆石症、胆总管结石、肝吸虫感染）是主要的危险因素。"},"tr":{"name":"Safra kesesi adenokarsinomu ve ekstrahepatik safra yolları","synonyms":["Safra kesesi adenokarsinomu ve EBT"]}},"xrefs":[{"source":"ICD-10","code":"C23","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C24.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C24.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C24.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018536","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707555","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":2.62,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}