{"orpha":424975,"name":"Squamous cell carcinoma of liver and intrahepatic biliary tract","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Squamous cell carcinoma of liver and IBT"],"definition":"Squamous cell carcinoma of liver and intrahepatic biliary tract is an extremely rare, primary, malignant liver and biliray tract epithelial tumor originating in the intrahepatic bile duct epithelium histologically characterized by the presence of keratinization and/or intracellular bridges. Patients typically present abdominal pain in the right upper quadrant, jaundice, nausea, vomiting, anorexia, weight loss, fever and/or dyspepsia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424975/","orphanet_url":"https://www.orpha.net/en/disease/detail/424975","names":{"fr":{"name":"Carcinome épidermoïde du foie et des voies biliaires intrahépatiques","synonyms":["Carcinome épidermoïde du foie et des VBIH"],"definition":"Tumeur épithéliale maligne rare du foie et des voies biliaires intrahépatiques prenant naissance dans l'épithélium des voies biliaires intrahépatiques, caractérisée sur le plan histologique par la présence de kératinisation et/ou de ponts intracellulaires. Les signes typiques sont des douleurs abdominales dans le quadrant supérieur droit, un ictère, des nausées, des vomissements, une anorexie, une perte de poids, une fièvre et/ou une dyspepsie."},"de":{"name":"Plattenepithelkarzinom der Leber und intrahepatischen Gallengänge","synonyms":["Squamöses Karzinom der Leber und intrahepatischen Gallengänge"]},"es":{"name":"Carcinoma de células escamosas de hígado y vías biliares intrahepáticas","synonyms":["Carcinoma carcinoide de hígado y VBI"],"definition":"Es un tumor epitelial primario de hígado y vías biliares, maligno y muy poco frecuente, que se origina en el epitelio del conducto biliar intrahepático. Histológicamente, está caracterizado por la presencia de queratinización y/o puentes intracelulares. Los afectados suelen presentar dolor abdominal en el cuadrante superior derecho, ictericia, náuseas, vómitos, anorexia, pérdida de peso, fiebre y/o dispepsia."},"it":{"name":"Carcinoma a cellule squamose del fegato e delle vie biliari intraepatiche","synonyms":["Carcinoma a cellule squamose del fegato e delle VBI"],"definition":"Il carcinoma a cellule squamose del fegato e delle vie biliari intraepatiche è una neoplasia epiteliale primitiva maligna molto rara del fegato e delle vie biliari, che origina dall'epitelio del dotto biliare intraepatico. Dal punto di vista istologico, è caratterizzato dalla presenza di cheratinizzazione e/o ponti intracellulari. Di solito i pazienti presentano dolore addominale nel quadrante superiore destro, ittero, nausea, vomito, anoressia, perdita di peso, febbre e/o dispepsia."},"nl":{"name":"Plaveiselcelcarcinoom van lever en intrahepatische galwegen","synonyms":["Plaveiselcelcarcinoom van lever en IBT"],"definition":"Plaveiselcelcarcinoom van de lever en intrahepatische galwegen is een uiterst zeldzame, primaire, maligne epitheliale tumor van de lever en galwegen die ontstaat in het epitheel van de intrahepatische galwegen en die histologisch gekarakteriseerd wordt door de aanwezigheid van keratinisatie en/of intracellulaire bruggen. Patiënten vertonen doorgaans buikpijn in het rechter bovenkwadrant, geelzucht, misselijkheid, braken, anorexie, gewichtsverlies, koorts en/of dyspepsie."},"pt":{"name":"Carcinoma de células escamosas hepático e do tracto biliar intra-hepático","synonyms":["Carcinoma de células escamosas do fígado e do TBIH"]},"pl":{"name":"Rak płaskonabłonkowy wątroby i wewnątrzwątrobowego przewodu żółciowego","synonyms":["Rak płaskonabłonkowy wątroby i IBT"],"definition":"*Rak płaskonabłonkowy wątroby i wewnątrzwątrobowych dróg żółciowych jest niezwykle rzadkim, pierwotnym, złośliwym nowotworem nabłonkowym wątroby i dróg żółciowych, wywodzącym się z nabłonka wewnątrzwątrobowego przewodu żółciowego, który histologicznie charakteryzuje się obecnością rogowacenia i/lub tworzeniem wewnątrzkomórkowych mostków. U chorych typowo występuje ból w prawym górnym kwadrancie brzucha, żółtaczka, nudności, wymioty, jadłowstręt, chudnięcie, gorączka i/lub objawy dyspeptyczne."},"cs":{"name":"Dlaždicobuněčný karcinom jater a intrahepatálních žlučových cest","synonyms":["Dlaždicobuněčný karcinom jater a IBT"]},"uk":{"name":"Плоскоклітинний рак печінки та внутрішньопечінкових жовчних шляхів","synonyms":["Плоскоклітинний рак печінки та IBT"],"definition":"Squamous cell carcinoma of liver and intrahepatic biliary tract is an extremely rare, primary, malignant liver and biliray tract epithelial tumor originating in the intrahepatic bile duct epithelium histologically characterized by the presence of keratinization and/or intracellular bridges. Patients typically present abdominal pain in the right upper quadrant, jaundice, nausea, vomiting, anorexia, weight loss, fever and/or dyspepsia."},"zh":{"name":"肝和肝内胆道鳞状细胞癌","synonyms":["肝鳞状细胞癌和IBT"],"definition":"肝及肝内胆管鳞状细胞癌是一种非常罕见的原发性恶性肝及胆管上皮性肿瘤，起源于肝内胆管上皮，其组织学特征是存在角化珠和/或细胞内桥。患者通常表现为右上腹疼痛、黄疸、恶心、呕吐、厌食、体重减轻、发热和/或消化不良。"},"tr":{"name":"Karaciğer ve intrahepatik safra yollarının skuamöz hücreli karsinomu","synonyms":["Karaciğer ve IBT'nin skuamöz hücreli karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C22.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C22.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018534","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4749400","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 / 1 000 000","value":0.009,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.016,"area":"Latvia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"area":"Lithuania","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Norway","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.005,"area":"Poland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.006,"area":"Portugal","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Slovakia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.017,"area":"Switzerland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.002,"area":"Netherlands","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.014,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.009,"area":"Austria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.003,"area":"Bulgaria","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.018,"area":"Czech Republic","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.009,"area":"Estonia","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.007,"area":"Finland","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.008,"area":"France","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.004,"area":"Germany","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.019,"area":"Italy","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}