{"orpha":99971,"name":"Well-differentiated liposarcoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["ALT","Atypical lipoma","Atypical lipomatous tumor","WDLS"],"definition":"Well-differentiated liposarcoma (WDLS), the most common type of liposarcoma (LS), is a slow growing, painless tumor usually located in the retroperitoneum or the limbs. It is composed of proliferating mature adipocytes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99971/","orphanet_url":"https://www.orpha.net/en/disease/detail/99971","names":{"fr":{"name":"Liposarcome bien différencié","synonyms":["ALT","Lipome atypique","Tumeur lipomateuse atypique","WDLS"],"definition":"Le liposarcome bien différencié (LBD), le type le plus commun de liposarcome (LS), est une tumeur indolore à croissance lente généralement localisée dans rétropéritoine ou les membres. Il se compose d'adipocytes matures proliférants."},"de":{"name":"Liposarkom, hochdifferenziertes","synonyms":["ALT","Lipom, atypisches","Tumor, atypischer lipomatöser","WDLS"],"definition":"Das hochdifferenzierte Liposarkom (WDLS), der häufigste Liposarkom-Typ (LS; siehe dort), ist ein langsam wachsender, schmerzfreier Tumor, der sich in der Regel im Retroperitoneum oder in den Extremitäten befindet. Es besteht aus proliferierenden reifen Adipozyten."},"es":{"name":"Liposarcoma bien diferenciado","synonyms":["ALT","Lipoma atípico","Tumor lipomatoso atípico","WDLS"],"definition":"El liposarcoma bien diferenciado (WDLS), el tipo de liposarcoma (LS; consulte este término) más frecuente, es un tumor indoloro de crecimiento lento localizado en el retroperitoneo o en las extremidades. Se compone de adipocitos maduros proliferativos."},"it":{"name":"Liposarcoma ben differenziato","synonyms":["ALT","Lipoma atipico","Tumore lipomatoso atipico","WDLS"],"definition":"Il liposarcoma ben differenziato (WDLS), la forma più comune di liposarcoma (LS; si veda questo termine), è un tumore indolore a progressione lenta, che si localizza di solito nel retroperitoneo o sugli arti. È formato da adipociti maturi proliferanti."},"nl":{"name":"Goed gedifferentieerd liposarcoom","synonyms":["ALT","Atypisch lipoom","Atypische lipomateuze tumor","WDLS"],"definition":"Goed gedifferentieerd liposarcoom (WDLS), het meest voorkomende type van liposarcoom (LS; zie deze term), is een traag groeiende, pijnloze tumor die meestal is gesitueerd in het retroperitoneum of de ledematen. Het bestaat uit prolifererende volgroeide adipocyten."},"pt":{"name":"Lipossarcoma bem diferenciado"},"pl":{"name":"Tłuszczakomięsak dobrze zróżnicowany","synonyms":["ALT","Atypowy guz tłuszczowy","Tłuszczak atypowy","WDLS"]},"cs":{"name":"Dobře diferencovaný liposarkom","synonyms":["ALT","Atypický lipom","Atypický lipomatózní nádor","WDLS"]},"uk":{"name":"Добре диференційована ліпосаркома","synonyms":["ALT","Атипова ліпома","Атипова ліпоматозна пухлина","WDLS"],"definition":"Well-differentiated liposarcoma (WDLS), the most common type of liposarcoma (LS; see this term), is a slow growing, painless tumor usually located in the retroperitoneum or the limbs. It is composed of proliferating mature adipocytes."},"zh":{"name":"高分化脂肪肉瘤","synonyms":["非典型脂肪性肿瘤"],"definition":"分化良好的脂肪肉瘤(WDLS)是最常见的脂肪肉瘤(LS)，是一种生长缓慢、无痛的肿瘤，通常位于腹膜后或四肢。它由增殖的成熟脂肪细胞组成。"},"tr":{"name":"İyi diferansiye liposarkom","synonyms":["ALT","WDLS","Atipik lipom","Atipik lipomatöz tümör"]}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"MONDO","code":"0005103","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1370889","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":69078,"name":"Liposarcoma","classifications":[202]}],"children":[],"genes":[{"symbol":"CDK4","name":"cyclin dependent kinase 4","gene_type":"gene with protein product","association":"Role in the phenotype of","status":"Assessed","source":null,"hgnc":"1773","ensembl":"ENSG00000135446","omim":"123829","loci":["12q14.1"]},{"symbol":"HMGA2","name":"high mobility group AT-hook 2","gene_type":"gene with protein product","association":"Role in the phenotype of","status":"Assessed","source":null,"hgnc":"5009","ensembl":"ENSG00000149948","omim":"600698","loci":["12q14.3"]},{"symbol":"MDM2","name":"MDM2 proto-oncogene","gene_type":"gene with protein product","association":"Role in the phenotype of","status":"Assessed","source":null,"hgnc":"6973","ensembl":"ENSG00000135679","omim":"164785","loci":["12q15"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.51,"area":"Europe","status":"Validated","source":"22517534[PMID]_[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012211","label":"Abnormal renal physiology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002579","label":"Gastrointestinal dysmotility","frequency":"Occasional (29-5%)","criteria":null}]}}