{"orpha":86896,"name":"Histiocytic sarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare histiocytic tumor characterized by a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Most cases occur in extranodal sites, most commonly the intestinal tract, skin, and soft tissue. Patients may present with a solitary mass, lymphadenopathy, a skin rash or numerous tumors on the trunk and extremities, lytic bone lesions, hepatosplenomegaly with pancytopenia, intestinal obstruction, and/or systemic symptoms. The neoplasm is aggressive with typically poor therapy response.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86896/","orphanet_url":"https://www.orpha.net/en/disease/detail/86896","names":{"fr":{"name":"Sarcome histiocytaire","definition":"Tumeur histiocytaire rare caractérisée par une prolifération maligne de cellules présentant des caractéristiques morphologiques et immunophénotypiques d'histiocytes tissulaires matures. Les sites extranodaux, le plus souvent le tractus intestinal, la peau et les tissus mous, sont affectés dans la plupart des cas. Les patients peuvent présenter une masse, une lymphadénopathie, une éruption cutanée ou de nombreuses tumeurs sur le tronc et les membres, des lésions osseuses lytiques, une hépatosplénomégalie avec pancytopénie, une occlusion intestinale et/ou des symptômes systémiques. Le néoplasme est agressif et la réponse thérapeutique est généralement faible."},"de":{"name":"Sarkom, histiozytäres","definition":"Ein seltener histiozytärer Tumor, der durch eine maligne Proliferation von Zellen gekennzeichnet ist, die morphologische und immunphänotypische Merkmale von reifen Gewebehistiozyten aufweisen. Die meisten Fälle treten extranodal auf, am häufigsten im Darmtrakt, in der Haut und im Weichteilgewebe. Die Patienten können sich mit einer solitären Gewebeverdichtung, Lymphadenopathie, einem Hautausschlag oder zahlreichen Tumoren an Rumpf und Extremitäten, lytischen Knochenläsionen, Hepatosplenomegalie mit Panzytopenie, Darmverschluss und/oder systemischen Symptomen vorstellen. Die Neoplasie ist aggressiv und spricht in der Regel schlecht auf die Therapie an."},"es":{"name":"Sarcoma histiocítico","definition":"Es un tumor histiocítico poco frecuente caracterizado por una proliferación maligna de células que presentan características morfológicas e inmunofenotípicas de los histiocitos de tejidos maduros. La mayoría de los casos ocurren en sitios extraganglionares, con mayor frecuencia en el tracto intestinal, la piel y los tejidos blandos. Los pacientes pueden presentarse con una masa solitaria, linfadenopatía, una erupción cutánea o numerosos tumores en el tronco y las extremidades, lesiones óseas líticas, hepatoesplenomegalia con pancitopenia, obstrucción intestinal y/o síntomas sistémicos. La neoplasia es agresiva con una respuesta terapéutica típicamente deficiente."},"it":{"name":"Sarcoma istiocitico","definition":"Si tratta di un tumore istiocitico raro, caratterizzato dalla proliferazione maligna di cellule con le caratteristiche morfologiche e immunofenotipiche degli istiociti dei tessuti maturi. La malattia si localizza per lo più nei siti extranodali, in genere nel tratto intestinale, sulla cute e nei tessuti molli. I pazienti presentano una massa solitaria, linfoadenopatia, eruzioni cutanee o tumori multipli sul tronco e sugli arti, lesioni osteolitiche, epatosplenomegalia con pancitopenia, ostruzione intestinale e/o sintomi sistemici. La neoplasia è aggressiva e risponde poco alla terapia."},"nl":{"name":"Histiocytair sarcoom","definition":"Een zeldzame histiocytaire tumor, gekarakteriseerd door een maligne proliferatie van cellen die morfologische en immunofenotypische kenmerken van rijpe histiocyten in weefsel vertonen. De meeste gevallen komen voor op extranodale plaatsen, meestal darmkanaal, huid, en weke delen. Patiënten kunnen zich presenteren met een solitaire massa, lymfadenopathie, huiduitslag of talloze tumoren op romp en ledematen, lytische botlaesies, hepatosplenomegalie met pancytopenie, darmobstructie, en/of systemische symptomen. Het neoplasma is agressief en vertoont doorgaans weinig respons op therapie."},"pt":{"name":"Sarcoma histiocítico"},"pl":{"name":"Mięsak histiocytarny"},"cs":{"name":"Histiocytární sarkom"},"uk":{"name":"Гістіоцитарна саркома","definition":"A rare histiocytic tumor characterized by a malignant proliferation of cells showing morphological and immunophenotypic features of mature tissue histiocytes. Most cases occur in extranodal sites, most commonly the intestinal tract, skin, and soft tissue. Patients may present with a solitary mass, lymphadenopathy, a skin rash or numerous tumors on the trunk and extremities, lytic bone lesions, hepatosplenomegaly with pancytopenia, intestinal obstruction, and/or systemic symptoms. The neoplasm is aggressive with typically poor therapy response."},"zh":{"name":"组织细胞肉瘤"},"tr":{"name":"Histiyositik sarkom"}},"xrefs":[{"source":"ICD-10","code":"C96.8","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2B31.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"911785965"},{"source":"MONDO","code":"0019479","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054747","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334663","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10076876","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":98288,"name":"Macrophage or histiocytic tumor","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}