{"orpha":555434,"name":"Fibrohistiocytic inflammatory pseudotumor of the liver","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":[],"definition":"A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with fibrohistiocytic infiltration (including xanthogranulomatous inflammation, multinucleated giant cells, and neutrophilic infiltration), typically localized in the peripheral hepatic parenchyma. Presentation may be of non-specific symptoms (fever, malaise, and abdominal pain) or as an incidental finding.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-555434/","orphanet_url":"https://www.orpha.net/en/disease/detail/555434","names":{"fr":{"name":"Pseudotumeur inflammatoire du foie, forme fibrohistiocytaire","definition":"Sous-type de pseudotumeur inflammatoire du foie caractérisé par une tumeur bénigne, bien circonscrite, avec une infiltration fibrohistiocytaire (comprenant une inflammation xanthogranulomateuse, des cellules géantes multinucléées et une infiltration neutrophile), typiquement localisée dans le parenchyme hépatique périphérique. La maladie peut se manifester sous forme de signes cliniques non spécifiques (fièvre, malaise et douleurs abdominales) ou être découverte de manière fortuite."},"de":{"name":"Fibrohistiozytischer inflammatorischer Pseudotumor der Leber","definition":"Ein Subtyp des entzündlichen Pseudotumors der Leber, der durch einen gutartigen, gut umschriebenen Tumor mit fibrohistiozytärer Infiltration (einschließlich xanthogranulomatöser Entzündung, vielkerniger Riesenzellen und neutrophiler Infiltration) gekennzeichnet ist und typischerweise im peripheren Leberparenchym lokalisiert ist. Die Erkrankung kann mit unspezifischen Symptomen (Fieber, Unwohlsein und Bauchschmerzen) oder als Zufallsbefund auftreten."},"es":{"name":"Pseudotumor inflamatorio fibrohistiocítico del hígado","definition":"Es un subtipo de pseudotumor inflamatorio hepático caracterizado por un tumor benigno y bien delimitado con infiltración fibrohistiocítica (incluyendo inflamación xantogranulomatosa, células gigantes multinucleadas e infiltración neutrofílica), localizado típicamente en el parénquima hepático periférico. Se puede presentar con síntomas inespecíficos (tales como fiebre, malestar y dolor abdominal) o como un hallazgo incidental."},"it":{"name":"Pseudotumore infiammatorio fibroistiocitario del fegato","definition":"Si tratta di un sottotipo di pseudotumore infiammatorio del fegato, che consiste in una massa benigna e ben delimitata con infiltrazione fibroistiocitaria (che comprende l'infiammazione xantogranulomatosa, le cellule giganti multinucleate e l'infiltrazione neutrofila), di solito localizzata nel parenchima epatico periferico. La malattia può esordire con sintomi aspecifici (febbre, malessere e dolore addominale), oppure può essere diagnosticata casualmente."},"nl":{"name":"Fibrohistiocytaire inflammatoire pseudotumor van lever","definition":"Een subtype van inflammatoire pseudotumor van lever, gekarakteriseerd door een goedaardige, goed begrensde tumor met fibrohistiocytaire infiltratie (inclusief xanthogranulomateuze inflammatie, multinucleaire reuzencellen, en neutrofiele infiltratie), doorgaans gesitueerd in perifeer leverparenchym. Het kan zich presenteren met niet-specifieke symptomen (koorts, malaise, en abdominale pijn) of toevallig ontdekt worden."},"pt":{"name":"Pseudotumor inflamatório fibrohistiocítico do fígado"},"pl":{"name":"Guz rzekomy fibrohistiocytarny zapalnywątroby"},"cs":{"name":"Fibrohistiocytický zánětlivý pseudotumor jater"},"uk":{"name":"Фіброгістіоцитарна запальна псевдопухлина печінки","definition":"A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with fibrohistiocytic infiltration (including xanthogranulomatous inflammation, multinucleated giant cells, and neutrophilic infiltration), typically localized in the peripheral hepatic parenchyma. Presentation may be of non-specific symptoms (fever, malaise, and abdominal pain) or as an incidental finding."},"zh":{"name":"肝纤维组织细胞炎性假瘤","definition":"肝脏炎症性假瘤的一种亚型，其特征是良性边界清晰的肿瘤，有纤维组织细胞浸润（包括黄色肉芽肿性炎症、多核巨细胞和中性粒细胞浸润），典型的定位在周围肝实质。表现为非特异性症状（发热、乏力和腹痛）或作为偶然发现。"},"tr":{"name":"Karaciğer fibrohistiyositik inflamatuar psödotümörü"}},"xrefs":[{"source":"ICD-10","code":"K75.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035312","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681328","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":90003,"name":"Inflammatory pseudotumor of the liver","classifications":[183]}],"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_17571078[PMID]"}],"phenotypes":null}