{"orpha":99969,"name":"Pleomorphic liposarcoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["PLS"],"definition":"Pleomorphic liposarcoma (PLS), the rarest subtype of liposarcoma (LS), is an aggressive, fast growing tumor located usually in the deep soft tissues of the lower and upper extremities. It is characterized by a variable number of pleomorphic lipoblasts and, in contrast to dedifferentiated liposarcoma, it lacks any association with well-differentiated liposarcoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99969/","orphanet_url":"https://www.orpha.net/en/disease/detail/99969","names":{"fr":{"name":"Liposarcome pléiomorphe","definition":"Le liposarcome pléiomorphe (LP), le plus rare sous-type de liposarcome (LS) est une tumeur agressive à croissance rapide généralement localisée dans les tissus mous profonds des membres inférieurs et supérieurs. Il est caractérisé par un nombre variable de lipoblastes pléomorphes et, contrairement au liposarcome dédifférencié, il n'est pas associé au liposarcome bien différencié."},"de":{"name":"Liposarkom, pleomorphes","synonyms":["PLS"],"definition":"Das pleomorphe Liposarkom (PLS), der seltenste Subtyp des Liposarkoms (LS; siehe dort), ist ein aggressiver, schnell wachsender Tumor, der sich in der Regel in den tiefen Weichteilen der unteren und oberen Extremitäten befindet. Er ist durch eine unterschiedliche Zahl von pleomorphen Lipoblasten gekennzeichnet und bei ihm fehlt, im Unterschied zum dedifferenzierten Liposarkom, jede Assoziierung mit dem hochdifferenzierten Liposarkom (siehe jeweils dort)."},"es":{"name":"Liposarcoma pleomórfico","definition":"El liposarcoma pleomórfico (PLS), el subtipo menos frecuente de liposarcoma (LS; consulte este término). Es un tumor agresivo de crecimiento rápido localizado normalmente en las partes blandas profundas de las extremidades inferiores y superiores. Se caracteriza por un número variable de lipoblastos pleomórficos y, en contraste con el liposarcoma desdiferenciado, carece de cualquier relación con el liposarcoma bien diferenciado (consulte estos términos)."},"it":{"name":"Liposarcoma pleomorfo","synonyms":["PLS"],"definition":"Il liposarcoma pleomorfo (PLS), il sottotipo di liposarcoma (LS; si veda questo termine) più raro, è un tumore aggressivo a progressione rapida, che si localizza di solito nei tessuti molli profondi degli arti inferiori e superiori. Si caratterizza per la presenza di un numero variabile di lipoblasti pleomorfi e, a differenza del liposarcoma dedifferenziato, non si associa al liposarcoma ben differenziato (si vedano questi termini)."},"nl":{"name":"Pleomorf liposarcoom","synonyms":["PLS","Pleiomorf liposarcoom"],"definition":"Pleomorf liposarcoom (PLS), het meest zeldzame subtype van liposarcoom (LS; zie deze term), is een agressieve en snelgroeiende tumor die doorgaans is gesitueerd de diepe weke delen van de onderste en bovenste ledematen. PLS wordt gekarakteriseerd door een variabel aantal pleomorfe lipoblasten en, in tegensteling tot gededifferentieerd liposarcoom, ontbreekt enige associatie met goed gedifferentieerd liposarcoom (zie deze termen)."},"pt":{"name":"Lipossarcoma pleomórfico"},"pl":{"name":"Tłuszczakomięsak pleomorficzny","synonyms":["plS"]},"cs":{"name":"Pleomorfní liposarkom","synonyms":["PLS"]},"uk":{"name":"Плеоморфна ліпосаркома","synonyms":["PLS"],"definition":"Pleomorphic liposarcoma (PLS), the rarest subtype of liposarcoma (LS; see this term), is an aggressive, fast growing tumor located usually in the deep soft tissues of the lower and upper extremities. It is characterized by a variable number of pleomorphic lipoblasts and, in contrast to dedifferentiated liposarcoma, it lacks any association with well-differentiated liposarcoma (see these terms)."},"zh":{"name":"多形性脂肪肉瘤","synonyms":["PLS"],"definition":"多形性脂肪肉瘤(PLS)是脂肪肉瘤(LS)最罕见的亚型，是一种侵袭性的、生长迅速的肿瘤，通常位于下肢和上肢的深部软组织。它的特点是多形性脂肪母细胞数量可变，与去分化脂肪肉瘤相比，它与分化良好的脂肪肉瘤没有任何关联(见该类词条)。"},"tr":{"name":"Pleomorfik liposarkom","synonyms":["PLS"]}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"MONDO","code":"0020562","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0205825","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073138","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":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"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":"2019-06-17","source":"10778771[PMID]","terms":[{"hpo":"HP:0012034","label":"Liposarcoma","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Very frequent (99-80%)","criteria":null}]}}