{"orpha":555437,"name":"Lymphoplasmacytic inflammatory pseudotumor of the liver","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["IgG4-related inflammatory pseudotumor of the liver"],"definition":"A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with diffuse lymphoplasmacytic infiltration with histological features of IgG4-related disease (numerous IgG4-positive plasma cells, prominent eosinophils, stromal fibrosis, fibroblastic proliferations and, frequently, obliterative phlebitis), and that is likely located around the hepatic hilum. Most often it is discovered as an incidental finding.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-555437/","orphanet_url":"https://www.orpha.net/en/disease/detail/555437","names":{"fr":{"name":"Pseudotumeur inflammatoire du foie, forme lymphoplasmocytaire","synonyms":["Pseudotumeur inflammatoire du foie associée à IgG4"],"definition":"Sous-type de pseudotumeur inflammatoire du foie caractérisée par une tumeur bénigne, bien circonscrite, avec une infiltration lymphoplasmocytaire diffuse et des caractéristiques histologiques de la maladie liée aux IgG4 (de nombreux plasmocytes IgG4-positifs, des éosinophiles proéminents, une fibrose stromale, des proliférations fibroblastiques et, souvent, une phlébite oblitérante), et qui est probablement située autour du hile hépatique. Dans la plupart des cas, la maladie est découverte de manière fortuite."},"de":{"name":"Lymphoplasmazytischer inflammatorischer Pseudotumor der Leber","synonyms":["IgG4-assoziierter inflammatorischer Pseudotumor der Leber"],"definition":"Ein Subtyp des entzündlichen Pseudotumors der Leber, der durch einen gutartigen, gut umschriebenen Tumor mit diffuser lymphoplasmatischer Infiltration mit histologischen Merkmalen einer IgG4-assoziierten Erkrankung (zahlreiche IgG4-positive Plasmazellen, prominente Eosinophile, stromale Fibrose, fibroblastische Proliferationen und häufig eine obliterative Phlebitis) gekennzeichnet ist und sich wahrscheinlich um den Leberhilus herum befindet. Meistens wird er als Zufallsbefund entdeckt."},"es":{"name":"Pseudotumor inflamatorio linfoplasmocítico del hígado","synonyms":["Pseudotumor inflamatorio del hígado asociado a IgG4","Pseudotumor inflamatorio hepático linfoplasmocítico"],"definition":"Es un subtipo de pseudotumor inflamatorio hepático caracterizado por un tumor benigno y bien delimitado con infiltración linfoplasmocítica difusa y hallazgos histológicos de enfermedad asociada a IgG4 (numerosas células plasmáticas IgG4 positivas, eosinofilia, fibrosis estromal, proliferación fibroblástica y, con frecuencia, flebitis obliterante), y localizado más frecuentemente alrededor del hilio hepático. En la mayoría de los casos se diagnostica como un hallazgo incidental."},"it":{"name":"Pseudotumore infiammatorio linfoplasmatico del fegato","definition":"Si tratta di un sottotipo di pseudotumore infiammatorio del fegato, che consiste in una massa benigna e ben delimitata con infiltrazione linfoplasmatica diffusa e con le caratteristiche istologiche tipiche della malattia correlata alle IgG4 (numerose plasmacellule positive per IgG4, evidenti eosinofili, fibrosi stromale, proliferazioni fibroblastiche e, spesso, flebite obliterativa), di solito localizzata attorno all'ilo epatico. Nella maggior parte dei pazienti viene diagnosticato casualmente."},"nl":{"name":"Lymfoplasmacytaire inflammatoire pseudotumor van lever","synonyms":["IgG4-gerelateerde inflammatoire pseudotumor van lever"],"definition":"Een subtype van inflammatoire pseudotumor van lever, gekarakteriseerd door een goedaardige, goed begrensde tumor met diffuse lymfoplasmacytaire infiltratie met histologische kenmerken van IgG4-gerelateerde ziekte (talrijke IgG4-positieve plasmacellen, prominent eosinofielen, stromale fibrose, fibroblastische proliferatie en, vaak, obliteratieve flebitis), doorgaans gesitueerd rond het leverhilum. Meestal wordt het toevallig ontdekt."},"pt":{"name":"Pseudotumor inflamatório linfoplasmocítico do fígado"},"pl":{"name":"Guz rzekomy limfoplazmatyczny zapalny wątroby","synonyms":["Guz rzekomy limfoplazmatyczny zapalny wątroby związany z IgG4"]},"cs":{"name":"Lymfoplazmocytický zánětlivý pseudotumor jater","synonyms":["IgG4-vázaný zánětlivý pseudotumor jater"]},"uk":{"name":"Лімфоплазмоцитарна запальна псевдопухлина печінки","synonyms":["Запальна псевдопухлина печінки, повязана з IgG4"],"definition":"A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with diffuse lymphoplasmacytic infiltration with histological features of IgG4-related disease (numerous IgG4-positive plasma cells, prominent eosinophils, stromal fibrosis, fibroblastic proliferations and, frequently, obliterative phlebitis), and that is likely located around the hepatic hilum. Most often it is discovered as an incidental finding."},"zh":{"name":"肝淋巴浆细胞性炎性假肿瘤","synonyms":["IgG4相关肝脏炎症性假瘤"],"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，有弥漫性淋巴细胞浸润，具有IgG4相关疾病的组织学特征（大量IgG4阳性浆细胞、主要为嗜酸性粒细胞、基质纤维化、纤维细胞增生，常有闭塞性静脉炎），可能位于肝门周围。大多数情况下，它是被偶然发现的。"},"tr":{"name":"Karaciğer lenfoplazmasitik inflamatuar psödotümörü","synonyms":["Karaciğerin IgG4 ile ilişkili inflamatuar psödotümörü"]}},"xrefs":[{"source":"ICD-10","code":"K75.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035313","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680170","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":284264,"name":"IgG4-related disease","classifications":[196]},{"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}