{"orpha":424970,"name":"Undifferentiated carcinoma of liver and intrahepatic biliary tract","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Undifferentiated carcinoma of liver and IBT"],"definition":"Undifferentiated carcinoma of liver and intrahepatic biliary tract is an extremely rare epithelial tumor of the liver and biliary tract which presents heterogenous histological findings and not yet fully defined clinicopathological characterisitcs. Patients usually present with nonspecific signs and symptoms, such as abdominal pain, nausea, vomiting, anorexia, weight loss and/or jaundice. Invasive growth, high metastatic potential and a rapid clinical course are typically associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-424970/","orphanet_url":"https://www.orpha.net/en/disease/detail/424970","names":{"fr":{"name":"Carcinome indifférencié du foie et des voies biliaires intrahépatiques","synonyms":["Carcinome indifférencié du foie et des VBIH"],"definition":"Tumeur épithéliale rare du foie et des voies biliaires intrahépatiques présentant une hétérogénéité histologique et dont les caractéristiques cliniques et pathologiques ne sont pas entièrement comprises. Les signes et les symptômes sont généralement non spécifiques, à savoir douleurs abdominales, nausées, vomissements, anorexie, perte de poids et/ou ictère. Une croissance invasive, un potentiel métastatique élevé et une évolution clinique rapide sont généralement associés."},"de":{"name":"Undifferenziertes Karzinom der Leber und intrahepatischen Gallengänge"},"es":{"name":"Carcinoma indiferenciado de hígado y vías biliares intrahepáticas","synonyms":["Carcinoma indiferenciado de hígado y VBI"],"definition":"Es un tumor epitelial muy poco frecuente del hígado y vías biliares que presenta hallazgos histológicos heterogéneos y características clínicopatológicas aún no completamente descritas. Los afectados suelen presentar signos y síntomas inespecíficos, tales como dolor abdominal, náuseas, vómitos, anorexia, pérdida de peso e/o ictericia. También se asocian habitualmente un crecimiento invasivo, elevado potencial metastásico y curso clínico rápido."},"it":{"name":"Carcinoma indifferenziato del fegato e delle vie biliari intraepatiche","synonyms":["Carcinoma non differenziato del fegato e delle VBI"],"definition":"Il carcinoma indifferenziato del fegato e delle vie biliari intraepatiche è un tumore epiteliale molto raro del fegato e delle vie biliari, caratterizzato da un quadro istologico eterogeneo e caratteristiche clinico-patologiche poco definite. Di solito i pazienti presentano segni clinici aspecifici, come il dolore addominale, la nausea, il vomito, l'anoressia, la perdita di peso e/o l'ittero. In genere il carcinoma è invasivo, ha un elevato potenziale metastatico ed un decorso clinico rapido."},"nl":{"name":"Ongedifferentieerd carcinoom van lever en intrahepatische galwegen","synonyms":["Ongedifferentieerd carcinoom van lever en IBT"],"definition":"Ongedifferentieerd carcinoom van de lever en intrahepatische galwegen is een uiterst zeldzame epitheliale tumor van de lever en galwegen die zich voordoet met heterogene histologische bevindingen en nog niet volledig gedefinieerde clinicopathologische kenmerken. Patiënten vertonen meestal niet-specifieke tekenen en symptomen, zoals buikpijn, misselijkheid, braken, anorexie, gewichtsverlies en/of geelzucht. Invasieve groei, hoog metastatisch potentieel en een snel klinisch verloop zijn doorgaans geassocieerd."},"pt":{"name":"Carcinoma hepático e do tracto biliar intra-hepático não diferenciado","synonyms":["Carcinoma indiferenciado do fígado e do TBIH"]},"pl":{"name":"Niezróżnicowany rak wątroby i wewnątrzwątrobowego przewodu żółciowego","synonyms":["Niezróżnicowany rak wątroby i IBT"],"definition":"*Niezróżnicowany rak wątroby i wewnątrzwątrobowych dróg żółciowych jest niezwykle rzadkim nabłonkowym nowotworem wątroby i dróg żółciowych, który wykazuje heterogenność histologiczną i nie jest w pełni scharakteryzowany pod względem klinicznym i patologicznym. U pacjentów występują zwykle niespecyficzne objawy przedmiotowe i podmiotowe, takie jak bóle brzucha, nudności, wymioty, jadłowstręt, chudnięcie i/lub żółtaczka. Zazwyczaj obserwuje się inwazyjny wzrost, duży potencjał przerzutowy i gwałtowny przebieg kliniczny."},"cs":{"name":"Nediferencovaný karcinom jater a intrahepatálních žlučových cest","synonyms":["Nediferencovaný karcinom jater a IBT"]},"uk":{"name":"Недиференційована карцинома печінки та внутрішньопечінкових жовчних шляхів","synonyms":["Недиференційована карцинома печінки та IBT"],"definition":"Undifferentiated carcinoma of liver and intrahepatic biliary tract is an extremely rare epithelial tumor of the liver and biliary tract which presents heterogenous histological findings and not yet fully defined clinicopathological characterisitcs. Patients usually present with nonspecific signs and symptoms, such as abdominal pain, nausea, vomiting, anorexia, weight loss and/or jaundice. Invasive growth, high metastatic potential and a rapid clinical course are typically associated."},"zh":{"name":"肝和肝内胆道未分化癌","synonyms":["肝未分化癌和IBT"],"definition":"肝及肝内胆管未分化癌是一种极为罕见的肝及胆管上皮性肿瘤，其组织学表现具有异质性，无法用临床病理检查结果明确诊断。患者通常表现为非特异性体征和症状，如腹痛、恶心、呕吐、厌食、体重减轻和/或黄疸。通常有侵袭性生长、高转移潜能和快速的临床进程。"},"tr":{"name":"Karaciğer ve intrahepatik safra yollarının farklılaşmamış karsinomu","synonyms":["Farklılaşmamış karaciğer karsinomu ve IBT"]}},"xrefs":[{"source":"ICD-10","code":"C22.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018533","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4749401","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":0.015,"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":0.011,"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":0.056,"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":0.046,"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":0.01,"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":0.02,"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":0.03,"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":0.043,"area":"Iceland","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":"Ireland","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]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.049,"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":0.033,"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":0.011,"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":0.013,"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":0.016,"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":0.098,"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":0.015,"area":"Spain","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.028,"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":0.005,"area":"United Kingdom","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}