{"orpha":99967,"name":"Myxoid/round cell liposarcoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["MRCLS"],"definition":"Myxoid/round cell liposarcoma (MRCLS) is a type of liposarcoma (LS) mostly located in the limbs, with a variable behavior depending on the histological subtype. Both myxoid and round cell are distinct histological subtypes of LS.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99967/","orphanet_url":"https://www.orpha.net/en/disease/detail/99967","names":{"fr":{"name":"Liposarcome myxoïde/à cellules rondes","synonyms":["MRCLS"],"definition":"Le liposarcome myxoïde/à cellules rondes (LMCR) est un type de liposarcome (LS) le plus souvent localisé dans les membres, avec des manifestations variables selon le sous-type histologique. Les cellules mixoïdes et rondes sont deux sous-types histologiques distincts de LS."},"de":{"name":"Liposarkom, myxoides/rundzelliges","synonyms":["MRCLS"],"definition":"Das myxoide/rundzellige Liposarkom (MRCLS) ist ein Typ des Liposarkoms (LS; siehe dort), das vor allem die Extremitäten betrifft und das je nach histologischem Subtyp ein unterschiedliches Verhalten zeigt. Sowohl das myxoide als auch das rundzellige Liposarkom sind distinkte histologische Subtypen des LS."},"es":{"name":"Liposarcoma mixoide/de células redondas","synonyms":["MRCLS"],"definition":"El liposarcoma mixoide/de células redondas (MRCLS) es un tipo de liposarcoma (LS; consulte este término) localizado principalmente en las extremidades, con un comportamiento variable dependiendo del subtipo histológico. Ambos, mixoide y de células redondas, son distintos subtipos histológicos de LS."},"it":{"name":"Liposarcoma mixoide/a cellule rotonde","synonyms":["MRCLS"],"definition":"Il liposarcoma mixoide/a cellule rotonde (MRCLS) è una forma di liposarcoma (LS; si veda questo termine), localizzata prevalentemente a livello degli arti, con un decorso variabile a seconda del sottotipo istologico. Sia le cellule mixoidi che quelle rotonde identificano sottotipi istologici distinti del LS."},"nl":{"name":"Myxoïd/rondcellig liposarcoom","synonyms":["MRCLS"],"definition":"Een type liposarcoom (LS), meestal gesitueerd in ledematen en met wisselvallig gedrag afhankelijk van het histologische subtype. Myxoïd liposarcoom en rondcellig liposarcoom zijn afzonderlijke subtypes van LS."},"pt":{"name":"Lipossarcoma mixóide"},"pl":{"name":"Tłuszczakomięsak śluzowaty/okrągłokomórkowy","synonyms":["MRCLS"]},"cs":{"name":"Myxoidní liposarkom","synonyms":["MRCLS"]},"uk":{"name":"Міксоїдна/круглоклітинна ліпосаркома","synonyms":["MRCLS"],"definition":"Myxoid/round cell liposarcoma (MRCLS) is a type of liposarcoma (LS; see this term) mostly located in the limbs, with a variable behavior depending on the histological subtype. Both myxoid and round cell are distinct histological subtypes of LS."},"zh":{"name":"黏液样/圆形细胞脂肪肉瘤","synonyms":["MRCLS"],"definition":"黏液样/圆形细胞脂肪肉瘤(MRCLS)是脂肪肉瘤的一种类型，主要位于四肢，其行为取决于组织学亚型。黏液样细胞和圆形细胞都是LS不同的组织学亚型。"},"tr":{"name":"Miksoid / yuvarlak hücreli liposarkom","synonyms":["MRCLS"]}},"xrefs":[{"source":"OMIM","code":"613488","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"MONDO","code":"0020561","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018208","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206634","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":"DDIT3","name":"DNA damage inducible transcript 3","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":null,"hgnc":"2726","ensembl":"ENSG00000175197","omim":"126337","loci":["12q13.3"]},{"symbol":"FUS","name":"FUS RNA binding protein","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":null,"hgnc":"4010","ensembl":"ENSG00000089280","omim":"137070","loci":["16p11.2"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1,"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:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002579","label":"Gastrointestinal dysmotility","frequency":"Occasional (29-5%)","criteria":null}]}}