{"orpha":529852,"name":"Combined hepatocellular carcinoma and cholangiocarcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Combined HCC-CC","Combined hepatocellular-cholangiocarcinoma","Hepatocholangiocarcinoma","cHCC-CC"],"definition":"A rare hepatic tumor characterized by the presence of both hepatocytic and cholangiocytic differentiation within a primary liver carcinoma. The lesion commonly arises in the context of chronic liver disease (such as hepatitis B or C, or steatohepatitis) or exposure to a variety of exogenous agents. Patients may present with signs and symptoms related to the tumor, as well as to the underlying condition. Typical manifestations include right upper quadrant abdominal pain, weight loss, hepatosplenomegaly, jaundice, and ascites. The entity has been associated with a worse prognosis than hepatocellular carcinoma after resection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-529852/","orphanet_url":"https://www.orpha.net/en/disease/detail/529852","names":{"fr":{"name":"Hépatocholangiocarcinome","synonyms":["Cholangiocarcinome hépatocellulaire combiné","HCC-CC combiné","cHCC-CC"],"definition":"Tumeur hépatique rare caractérisée par la présence d'une tumeur hépatique à différentiation hépatocellulaire et cholangiocarcinomateuse. La lésion survient généralement dans le contexte d'une maladie chronique du foie (telle que l'hépatite B ou C, ou la stéatohépatite) ou d'une exposition à divers facteurs exogènes. Les patients peuvent présenter des signes et des symptômes liés à la tumeur, ainsi qu'à la maladie sous-jacente. Les signes cliniques typiques sont des douleurs abdominales dans le quadrant supérieur droit, une perte de poids, une hépatosplénomégalie, un ictère et une ascite. La maladie a été associée à un pronostic plus défavorable par rapport au carcinome hépatocellulaire après résection."},"de":{"name":"Kombiniertes hepatozelluläres Karzinom und Cholangiokarzinom","synonyms":["Hepatocholangiokarzinom","Kombiniertes HCC-CC","Kombiniertes hepatozelluläres und Cholangiokarzinom","cHCC-CC"],"definition":"Ein seltener Lebertumor, der sich durch das Vorhandensein sowohl hepatozytärer als auch cholangiozytärer Differenzierung innerhalb eines primären Leberkarzinoms auszeichnet. Die Läsion entsteht häufig im Zusammenhang mit einer chronischen Lebererkrankung (z. B. Hepatitis B oder C oder Steatohepatitis) oder einer Exposition gegenüber einer Vielzahl von exogenen Substanzen. Die Patienten können Anzeichen und Symptome aufweisen, die sowohl mit dem Tumor als auch mit der Grunderkrankung zusammenhängen. Zu den typischen Symptomen gehören Bauchschmerzen im rechten oberen Quadranten, Gewichtsverlust, Hepatosplenomegalie, Gelbsucht und Aszites. Diese Entität ist nach der Resektion mit einer schlechteren Prognose verbunden als das hepatozelluläre Karzinom."},"es":{"name":"Carcinoma hepatocelular y colangiocarcinoma combinado","synonyms":["HCC-CC combinado","Hepatocarcinoma-colangiocarcinoma combinado","Hepatocolangiocarcinoma","cHCC-CC"],"definition":"Es un tumor hepático poco frecuente caracterizado por la presencia de diferenciación tanto hepatocítica como colangiocítica en un carcinoma primario de hígado. La lesión suele ocurrir en el contexto de una enfermedad hepática crónica (como la hepatitis B o C, o la esteatohepatitis) o por exposición a diversos agentes exógenos. Los pacientes pueden presentarse con signos y síntomas asociados al tumor, así como con la afección subyacente. Las manifestaciones típicas incluyen dolor abdominal en el cuadrante superior derecho, pérdida de peso, hepatoesplenomegalia, ictericia y ascitis. La entidad asocia un peor pronóstico que el carcinoma hepatocelular después de la resección."},"it":{"name":"Carcinoma epatocellulare combinato e colangiocarcinoma","definition":"Si tratta di un tumore epatico raro, caratterizzato dalla differenziazione sia epatocitica che colangiocitica all'interno di un carcinoma epatico primitivo. La lesione in genere insorge nel contesto di un'epatopatia cronica (ad es. l'epatite B o C, o la steatoepatite) o di un'esposizione a diversi agenti esogeni. I pazienti presentano i sintomi correlati al tumore e alla malattia preesistente. I segni clinici caratteristici comprendono il dolore nel quadrante superiore destro dell'addome, la perdita di peso, l'epatosplenomegalia, l'ittero e l'ascite. La prognosi è peggiore rispetto a quella del carcinoma epatocellulare dopo la resezione."},"nl":{"name":"Gecombineerd hepatocellulair carcinoom en cholangiocarcinoom","synonyms":["Gecombineerd HCC-CC","Gecombineerd hepatocellulair-cholangiocarcinoom","Hepatocholangiocarcinoom","cHCC-CC"],"definition":"Een zeldzame tumor van lever, gekarakteriseerd door de aanwezigheid van zowel hepatocytaire als cholangiocytaire differentiatie in een primair carcinoom van lever. De laesie ontstaat doorgaans in de context van chronische leverziekte (zoals hepatitis B of C, of steatohepatitis) of blootstelling aan een verscheidenheid van exogene agentia. Patiënten kunnen zich presenteren met verschijnselen en symptomen gerelateerd aan de tumor, alsook aan de onderliggende aandoening. Typische manifestaties zijn onder meer pijn in rechter bovenkwadrant van abdomen, gewichtsverlies, hepatosplenomegalie, geelzucht, en ascites. De entiteit werd geassocieerd met een slechtere prognose dan hepatocellulair carcinoom na excisie."},"pt":{"name":"Carcinoma hepatocelular e colangiocarcinoma combinado","synonyms":["HCC-CC combinado","Hepatocolangiocarcinoma","cHCC-CC"]},"pl":{"name":"Rak złożony wątrobowo-komórkowy i dróg żółciowych","synonyms":["Combined HCC-CC","Combined hepatocellular-cholangiocarcinoma","Hepatocholangiocarcinoma","cHCC-CC"]},"cs":{"name":"Kombinovaný hepatocelulární karcinom a cholangiokarcinom","synonyms":["cHCC-CC","Kombinované HCC-CC","Hepatocholangiokarcinom"]},"uk":{"name":"Поєднання гепатоцелюлярної карциноми та холангіокарциноми","synonyms":["cHCC-CC","Комбінований HCC-CC","Комбінована гепатоцелюлярно-холангіокарцинома","Гепатохолангіокарцинома"],"definition":"A rare hepatic tumor characterized by the presence of both hepatocytic and cholangiocytic differentiation within a primary liver carcinoma. The lesion commonly arises in the context of chronic liver disease (such as hepatitis B or C, or steatohepatitis) or exposure to a variety of exogenous agents. Patients may present with signs and symptoms related to the tumor, as well as to the underlying condition. Typical manifestations include right upper quadrant abdominal pain, weight loss, hepatosplenomegaly, jaundice, and ascites. The entity has been associated with a worse prognosis than hepatocellular carcinoma after resection."},"zh":{"name":"肝细胞癌合并胆管上皮癌","synonyms":["Combined hepatocellular-cholangiocarcinoma"]},"tr":{"name":"Kombine hepatoselüler karsinom ve kolanjiyokarsinom","synonyms":["cHCC-CC","Kombine HCC-CC","Hepatokolanjiokarsinom","Kombine hepatoselüler-kolanjiokarsinom"]}},"xrefs":[{"source":"ICD-10","code":"C22.0","qualifier":"BTNT","icd_relation":"inclusion","validated":true},{"source":"ICD-10","code":"C22.1","qualifier":"BTNT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2C12.00","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1097637010"},{"source":"MONDO","code":"0044791","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0221287","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]},{"orpha":424936,"name":"Carcinoma of liver and intrahepatic biliary tract","classifications":[183,202]},{"orpha":506210,"name":"Rare disorder potentially indicated for liver transplant","classifications":[233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_23034166[PMID]"}],"phenotypes":null}