{"orpha":386,"name":"Hepatic cystic hamartoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Biliary hamartoma","MHL","Mesenchymal hamartoma of liver","VMC","Von Meyenburg complexes disease"],"definition":"Hepatic cystic hamartoma, also named Mesenchyma hamartoma of liver, is a rare benign liver tumor of childhood, usually before the age of 2, of mesenchymal origin and variable clinical presentation (abdominal dissension, abdominal mass, pain, vomiting and signs of inferior vena cava compression).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-386/","orphanet_url":"https://www.orpha.net/en/disease/detail/386","names":{"fr":{"name":"Hamartome kystique hépatique","synonyms":["Hamartome biliaire","Hamartome mésenchymateux du foie","MHL","Maladie des complexes de von Meyenburg","VMC"],"definition":"L'hamartome kystique hépatique, aussi connu sous le nom d'hamartome mésenchymateux du foie, est une tumeur hépatique de l'enfant, rare et bénigne, apparaissant généralement avant l'âge de 2 ans. Il est d'origine mésenchymateuse et a une présentation clinique variable (distension abdominale, masse abdominale, douleurs, vomissements et signes de compression de la veine cave inférieure)."},"de":{"name":"Zystisches Leberhamartom","synonyms":["Biliäre Hamartome","Leberhamartom, mesenchymales","MHL","VMC","Von-Meyenburg-Komplex"],"definition":"Das zystische Leberhamartom, auch mesenchymales Leberhamartom genannt, ist ein seltener gutartiger Lebertumor des Kindesalters. In der Regel tritt er vor dem Alter von zwei Jahren auf, ist mesenchymalen Ursprungs und hat unterschiedliche klinische Symptome (aufgetriebenes Abdomen, abdomineller Tumor, Schmerzen, Erbrechen und Zeichen einer Kompression der Vena cava inferior)."},"es":{"name":"Hamartoma quístico hepático","synonyms":["Enfermedad de los complejos de Von Meyenburg","HMH","Hamartoma biliar","Hamartoma mesenquimal hepático","VMC"],"definition":"El hamartoma quístico hepático, también llamado hamartoma mesenquimal hepático, es un tumor hepático benigno poco frecuente que se presenta en la infancia, normalmente antes de los 2 años de edad, de origen mesenquimal y presentación clínica variable (disensión abdominal, masa abdominal, dolor, vómitos y signos de compresión de la vena cava inferior)."},"it":{"name":"Amartoma cistico epatico","synonyms":["Amartoma biliare","Amartoma mesenchimale del fegato","MHL","Malattia dei complessi di von Meyenburg","VMC"],"definition":"L'amartoma cistico epatico, noto anche come amartoma mesenchimale del fegato, è un tumore epatico benigno raro, che interessa l'infanzia, ad origine mesenchimale. L'esordio di solito si verifica prima dei due anni, con un quadro clinico variabile (distensione addominale, presenza di una massa nell'addome, dolore, vomito e segni di compressione della vena cava inferiore)."},"nl":{"name":"Hepatisch cystisch hamartoom","synonyms":["Biliair hamartoom","MHL","Mesenchymaal hamartoom van lever","VMC","Ziekte van von Meyenburg-complexen"],"definition":"Hepatisch cystisch hamartoom, ook gekend als mesenchymaal hamartoom van lever, is een zeldzame goedaardige tumor van lever in de kindertijd, meestal voor de leeftijd van 2 jaar, van mesenchymale oorsprong en met variabele klinische presentatie (gezwollen abdomen, abdominale massa, pijn, braken, en verschijnselen van compressie van vena cava inferior)."},"pt":{"name":"Hamartoma hepático quístico"},"pl":{"name":"Torbielowaty guz wątroby o utkaniu hamartoma","synonyms":["Choroba kompleksów Von Meyenburga","Hamartoma dróg żółciowych","Mezenchymalny guz wątroby o utkaniu hamartoma","MHL","VMC"]},"cs":{"name":"Hepatální cystický hamartom","synonyms":["Biliární hamartom","MHL","Mesenchymový hamartom jater","VMC","Nemoc von Meyenbergova komplexu"]},"uk":{"name":"Кістозна гамартома печінки","synonyms":["Біліарна гамартома","Мезенхімальна гамартома печінки","MHL","VMC","Хвороба комплексів фон Мейєнбурга"],"definition":"Hepatic cystic hamartoma, also named Mesenchyma hamartoma of liver, is a rare benign liver tumor of childhood, usually before the age of 2, of mesenchymal origin and variable clinical presentation (abdominal dissension, abdominal mass, pain, vomiting and signs of inferior vena cava compression)."},"zh":{"name":"肝脏胆囊错构瘤","synonyms":["Von Meyenburg综合征"]},"tr":{"name":"Karaciğer kistik hamartom","synonyms":["MHL","VMC","Biliyer hamartom","Karaciğerin mezenkimal hamartomu","Von Meyenburg kompleks hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"D13.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E92.7","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1416010483"},{"source":"MONDO","code":"0018308","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333971","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2651","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10074610","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":306636,"name":"Rare tumor of liver and intrahepatic biliary tract","classifications":[183,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Infancy"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":260.0,"area":"Worldwide","status":"Validated","source":"26858497[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"26858497[PMID]_ORPHANET"}],"phenotypes":null}