{"orpha":566841,"name":"Liver adenomatosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Hepatic adenomatosis"],"definition":"A rare neoplastic disease characterized by the presence of ten or more hepatocellular adenomas in a background of normal appearing hepatic parenchyma. The majority of reported cases are female. There is no association with steroid use. The condition is considered benign, although the risk of complications (such as malignant transformation or spontaneous rupture with intraperitoneal hemorrhage) is much higher than in isolated hepatic adenoma. Hepatocellular carcinoma develops in less than 10% of cases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-566841/","orphanet_url":"https://www.orpha.net/en/disease/detail/566841","names":{"fr":{"name":"Adénomatose hépatique","synonyms":["Adénomatose du foie"],"definition":"Maladie néoplasique rare caractérisée par la présence de dix adénomes hépatocellulaires ou plus dans un contexte de parenchyme hépatique d'apparence normale. La majorité des cas rapportés sont des femmes. Il n'existe pas de lien avec l'utilisation de stéroïdes. La maladie est considérée comme bénigne, bien que le risque de complications (telles qu'une transformation maligne ou une rupture spontanée avec hémorragie intrapéritonéale) soit beaucoup plus élevé que dans le cas d'un adénome hépatique isolé. Le carcinome hépatocellulaire se développe dans moins de 10 % des cas."},"de":{"name":"Adenomatose der Leber","synonyms":["Hepatische Adenomatose"],"definition":"Eine seltene Neoplasie, die durch das Vorhandensein von zehn oder mehr hepatozellulären Adenomen vor dem Hintergrund eines normal erscheinenden Leberparenchyms gekennzeichnet ist. Die Mehrzahl der gemeldeten Fälle betrifft Frauen. Es besteht kein Zusammenhang mit der Einnahme von Steroiden. Die Erkrankung gilt als gutartig, obwohl das Risiko von Komplikationen (wie z. B. maligne Transformation oder spontane Ruptur mit intraperitonealer Blutung) viel höher ist als bei isolierten hepatozellulären Adenomen. Ein hepatozelluläres Karzinom entwickelt sich in weniger als 10 % der Fälle."},"es":{"name":"Adenomatosis hepática","synonyms":["Adenomatosis de hígado"],"definition":"Es una enfermedad neoplásica poco frecuente caracterizada por la presencia de diez o más adenomas hepatocelulares en un parénquima hepático de apariencia normal. La mayoría de los casos descritos son mujeres. No existe asociación con el uso de esteroides. El trastorno se considera benigno, aunque el riesgo de complicaciones (tales como la transformación maligna o una ruptura espontánea con hemorragia intraperitoneal) es mucho mayor que en el adenoma hepático aislado. El carcinoma hepatocelular se desarrolla en menos del 10% de los casos."},"it":{"name":"Adenomatosi del fegato","synonyms":["Adenomatosi epatica"],"definition":"Si tratta di una neoplasia rara, caratterizzata dalla presenza di dieci o più adenomi epatocellulari localizzati nel parenchima epatico che peraltro presenta un aspetto normale. La malattia colpisce per lo più le femmine e non si associa all'assunzione di steroidi. La malattia è considerata benigna, anche se la probabilità di complicanze (ad es. evoluzione maligna o rottura spontanea con emorragia intraperitoneale) è molto più elevata rispetto all'adenoma epatico isolato. Meno del 10% dei pazienti sviluppa un carcinoma epatocellulare."},"nl":{"name":"Adenomatose van lever","synonyms":["Hepatische adenomatose"],"definition":"Een zeldzame neoplastische aandoening, gekarakteriseerd door aanwezigheid van tien of meer hepatocellulaire adenomen tegen een achtergrond van normaal uitziend leverparenchym. De meerderheid van de gerapporteerde gevallen is vrouwelijk. Er is geen associatie met gebruik van steroïden. De ziekte wordt als goedaardig beschouwd, hoewel het risico op complicaties (zoals maligne transformatie of spontane ruptuur met intraperitoneale hemorragie) veel hoger ligt dan bij geïsoleerd adenoom van lever. Hepatocellulair carcinoom ontwikkelt bij minder dan 10% van de gevallen."},"pt":{"name":"Adenomatose hepática"},"pl":{"name":"Gruczolakowatość wątroby","synonyms":["Hepatic adenomatosis"]},"cs":{"name":"Adenomatóza jater","synonyms":["Hepatic adenomatosis"]},"uk":{"name":"Аденоматоз печінки","synonyms":["Аденоматоз печінки"],"definition":"A rare neoplastic disease characterized by the presence of ten or more hepatocellular adenomas in a background of normal appearing hepatic parenchyma. The majority of reported cases are female. There is no association with steroid use. The condition is considered benign, although the risk of complications (such as malignant transformation or spontaneous rupture with intraperitoneal hemorrhage) is much higher than in isolated hepatic adenoma. Hepatocellular carcinoma develops in less than 10% of cases."},"zh":{"name":"肝腺瘤病","synonyms":["Hepatic adenomatosis"]},"tr":{"name":"Karaciğer adenomatozu","synonyms":["Hepatik adenomatoz"]}},"xrefs":[{"source":"ICD-10","code":"D13.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E92.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035447","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680136","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":506210,"name":"Rare disorder potentially indicated for liver transplant","classifications":[233]},{"orpha":306636,"name":"Rare tumor of liver and intrahepatic biliary tract","classifications":[183,202]}],"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_26011213[PMID]"}],"phenotypes":null}