{"orpha":99970,"name":"Dedifferentiated liposarcoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["DDLS"],"definition":"Dedifferentiated liposarcoma (DDLS) is a high-grade subtype of liposarcoma (LS) that progresses from well-differentiated liposarcoma (WDLS), and most often occurs in the retroperitoneum. It is defined as a region of nonlipogenic sarcoma associated with WDLS. .","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99970/","orphanet_url":"https://www.orpha.net/en/disease/detail/99970","names":{"fr":{"name":"Liposarcome dédifférencié","synonyms":["DDLS"],"definition":"Le liposarcome dédifférencié est un sous-type de liposarcome de haut-grade, qui se développe à partir d'un liposarcome bien différencié, généralement dans le rétropéritoine. Il est défini comme une zone de sarcome non lipogénique associée à un liposarcome bien différencié."},"de":{"name":"Liposarkom, dedifferenziertes","synonyms":["DDLS"],"definition":"Das dedifferenzierte Liposarkom (DDLS) ist ein niedrig-maligner Subtyp des Liposarkoms (LS; siehe dort), der vom hochdifferenziertem Liposarkom (WDLS; siehe dort) ausgeht und am häufigsten im Retroperitoneum auftritt. In dieser Region werden nicht-lipogene Sarkome mit dem WDLS assoziiert gefunden."},"es":{"name":"Liposarcoma desdiferenciado","synonyms":["DDLS"],"definition":"El liposarcoma desdiferenciado (DDLS) es un subtipo de liposarcoma (LS; consulte este término) de alto grado, que progresa desde un liposarcoma bien diferenciado (WDLS; consulte este término) y que se produce con más frecuencia en el retroperitoneo. Se define como una región de sarcoma no lipogénico asociado con WDLS."},"it":{"name":"Liposarcoma dedifferenziato","synonyms":["DDLS"],"definition":"Il liposarcoma dedifferenziato (DDLS) è un sottotipo di liposarcoma (LS; si veda questo termine) di grado elevato, che origina dall'evoluzione del liposarcoma ben differenziato (WDLS; si veda questo termine), e insorge spesso nel retroperitoneo. Viene definito come un tessuto sarcomatoso non lipogenico associato a WDLS."},"nl":{"name":"Gededifferentieerd liposarcoom","synonyms":["DDLS"],"definition":"Gededifferentieerd liposarcoom (DDLS) is een subtype van liposarcoom (LS; zie deze term) met hoge graad dat voorkomt uit goed gedifferentieerd liposarcoom (WDLS; zie deze term) en het vaakst voorkomt in het retroperitoneum. Het wordt gedefinieerd als een gebied van niet-lipogeen sarcoom geassocieerd met WDLS."},"pt":{"name":"Lipossarcoma com perda de diferenciação"},"pl":{"name":"Tłuszczakomięsak odróżnicowany","synonyms":["DDLS"]},"cs":{"name":"Dediferencovaný liposarkom","synonyms":["DDLS"]},"uk":{"name":"Дедиференційована ліпсаркома","synonyms":["DDLS"],"definition":"Dedifferentiated liposarcoma (DDLS) is a high-grade subtype of liposarcoma (LS; see this term) that progresses from well-differentiated liposarcoma (WDLS; see this term), and most often occurs in the retroperitoneum. It is defined as a region of nonlipogenic sarcoma associated with WDLS. ."},"zh":{"name":"去分化脂肪肉瘤","synonyms":["DDLS"],"definition":"去分化脂肪肉瘤(DDLS)是脂肪肉瘤的一种高级别亚型，由分化良好的脂肪肉瘤（LS；见该词条）发展而来，最常发生在腹膜后。它被定义为与WDLS相关的非脂源性肉瘤区域。"},"tr":{"name":"Dediferansiye liposarkom","synonyms":["DDLS"]}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"MONDO","code":"0020563","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0205824","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073135","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":0.27,"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":null}