{"orpha":90003,"name":"Inflammatory pseudotumor of the liver","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare benign liver tumor characterized by a prominent inflammatory infiltrate and often mimicking a malignant liver neoplasm. The tumor is frequently solitary with a predilection for the right lobe; however, multiple lesions are possible. There are two clinicopathological subtypes: fibrohistiocytic inflammatory pseudotumor of the liver and lymphoplasmacytic inflammatory pseudotumor of the liver. Patients present with non-specific clinical symptoms such as abdominal pain or discomfort, fever, and weight loss. The condition may be associated with other chronic inflammatory or autoimmune diseases.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90003/","orphanet_url":"https://www.orpha.net/en/disease/detail/90003","names":{"fr":{"name":"Pseudotumeur inflammatoire du foie","definition":"Tumeur hépatique bénigne rare caractérisée par un infiltrat inflammatoire proéminent et imitant souvent une tumeur hépatique maligne. La tumeur est souvent solitaire avec une prédilection pour le lobe droit ; cependant, des lésions multiples sont possibles. Il existe deux sous-types clinicopathologiques : la pseudotumeur inflammatoire fibrohistiocytaire du foie et la pseudotumeur inflammatoire lymphoplasmocytaire du foie. Les patients présentent des signes cliniques non spécifiques, tels que des douleurs ou une gêne abdominale, de la fièvre et une perte de poids. La maladie peut être associée à d'autres maladies inflammatoires ou auto-immunes chroniques."},"de":{"name":"Pseudotumor der Leber, inflammatorischer","definition":"Ein seltener gutartiger Lebertumor, der durch ein ausgeprägtes entzündliches Infiltrat gekennzeichnet ist und oft eine bösartige Neoplasie der Leber imitiert. Der Tumor tritt häufig solitär auf, bevorzugt im rechten Lappen; es sind jedoch auch multiple Läsionen möglich. Es gibt zwei klinisch-pathologische Subtypen: den fibrohistiozytären entzündlichen Pseudotumor der Leber und den lymphoplasmazytischen entzündlichen Pseudotumor der Leber. Die Patienten zeigen unspezifische klinische Symptome wie Bauchschmerzen oder Unwohlsein, Fieber und Gewichtsverlust. Die Erkrankung kann mit anderen chronischen Entzündungs- oder Autoimmunerkrankungen einhergehen."},"es":{"name":"Pseudotumor inflamatorio del hígado","definition":"Es un tumor hepático benigno poco frecuente caracterizado por un prominente infiltrado inflamatorio que a menudo se asemeja a una neoplasia hepática maligna. Suele tratarse de un tumor solitario con predilección por el lóbulo derecho; sin embargo, también son posibles las lesiones múltiples. Existen dos subtipos clinicopatológicos: el pseudotumor inflamatorio fibrohistiocítico del hígado y pseudotumor inflamatorio linfoplasmocítico del hígado. Los pacientes presentan síntomas clínicos inespecíficos como dolor o malestar abdominal, fiebre y pérdida de peso. La enfermedad puede estar asociada a otras enfermedades inflamatorias o autoinmunes crónicas."},"it":{"name":"Pseudotumore infiammatorio del fegato","definition":"Si tratta di un tumore benigno raro del fegato, caratterizzato da un significativo infiltrato infiammatorio, che spesso assomiglia a una neoplasia epatica maligna. In genere il tumore è solitario e predilige il lobo destro; tuttavia, sono possibili lesioni multiple. Esistono due sottotipi clinico-patologici: lo pseudotumore infiammatorio fibroistiocitico del fegato e lo pseudotumore infiammatorio linfoplasmacitico del fegato. I pazienti presentano sintomi clinici aspecifici, come il dolore o la sensazione di fastidio addominale, la febbre e la perdita di peso. La malattia può associarsi ad altre malattie infiammatorie croniche o autoimmuni."},"nl":{"name":"Inflammatoire pseudotumor van lever","definition":"Een zeldzame goedaardige tumor van lever, gekenmerkt door een prominent inflammatoir infiltraat en vaak gelijkend op een maligne neoplasma van lever. De tumor is vaak solitair met een voorkeur voor de rechter leverkwab; multipele laesies zijn echter mogelijk. Er zijn twee clinicopathologische subtypes: fibrohistiocytaire inflammatoire pseudotumor van lever en lymfoplasmacytaire inflammatoire pseudotumor van lever. Patiënten presenteren zich met aspecifieke klinische symptomen zoals abdominale pijn or ongemak, koorts, en gewichtsverlies. De aandoening kan geassocieerd zijn met andere chronische inflammatoire aandoeningen of auto-immuunziekten."},"pt":{"name":"Pseudotumor inflamatório do fígado"},"pl":{"name":"Pseudoguz zapalny wątroby"},"cs":{"name":"IgG4 asociovaná hepatopatie","synonyms":["Zánětlivý pseudotumor jater"]},"uk":{"name":"Запальна псевдопухлина печінки","definition":"A rare benign liver tumor characterized by a prominent inflammatory infiltrate and often mimicking a malignant liver neoplasm. The tumor is frequently solitary with a predilection for the right lobe; however, multiple lesions are possible. There are two clinicopathological subtypes: fibrohistiocytic inflammatory pseudotumor of the liver and lymphoplasmacytic inflammatory pseudotumor of the liver. Patients present with non-specific clinical symptoms such as abdominal pain or discomfort, fever, and weight loss. The condition may be associated with other chronic inflammatory or autoimmune diseases."},"zh":{"name":"肝炎性假瘤"},"tr":{"name":"Karaciğerin inflamatuar psödotümörü"}},"xrefs":[{"source":"ICD-10","code":"K75.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"DB99.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1364737590"},{"source":"MONDO","code":"0019528","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1333967","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":57146,"name":"Rare hepatic disease"},"parents":[{"orpha":101939,"name":"Rare parenchymal liver disease","classifications":[183]}],"children":[{"orpha":555434,"name":"Fibrohistiocytic inflammatory pseudotumor of the liver","type":"Clinical subtype"},{"orpha":555437,"name":"Lymphoplasmacytic inflammatory pseudotumor of the liver","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":140.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-02-09","source":"29291783(PMID]_25663462[PMID]","terms":[{"hpo":"HP:0031140","label":"Abnormal liver sonography","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0002896","label":"Neoplasm of the liver","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030057","label":"Autoimmune antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025406","label":"Asthenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001080","label":"Biliary tract abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001394","label":"Cirrhosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0410369","label":"Increased hepatitis B virus antibody level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031964","label":"Elevated circulating alanine aminotransferase concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0031956","label":"Elevated circulating aspartate aminotransferase concentration","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006254","label":"Elevated alpha-fetoprotein","frequency":"Excluded (0%)","criteria":null}]}}