{"orpha":293202,"name":"Epithelioid sarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, soft tissue tumor characterized by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-293202/","orphanet_url":"https://www.orpha.net/en/disease/detail/293202","names":{"fr":{"name":"Sarcome épithélioïde","definition":"Tumeur rare des tissus mous caractérisée par un taux élevé de récidive locale, une atteinte des ganglions lymphatiques régionaux et des métastases à distance. Elle touche généralement les tissus mous sous-cutanés des doigts, de la main, de l'avant-bras, de la partie inférieure de la jambe ou du pied, et moins fréquemment d'autres parties du corps."},"de":{"name":"Epitheloidsarkom","definition":"Ein seltener Weichteiltumor, der sich durch eine hohe Inzidenz von Lokalrezidiven, regionalem Lymphknotenbefall und Fernmetastasen auszeichnet. Er befällt in der Regel das Weichteilgewebe unter der Haut von Finger, Hand, Unterarm, Unterschenkel oder Fuß, seltener auch andere Körperregionen."},"es":{"name":"Sarcoma epitelioide","definition":"Es un tumor de tejidos blandos poco frecuente, caracterizado por una alta incidencia de recidiva local, afectación de ganglios linfáticos regionales y metástasis a distancia. Afecta habitualmente al tejido blando que subyace la piel de dedos, manos, antebrazos, la parte inferior de la pierna o el pie y, con menor frecuencia, de otras áreas corporales."},"it":{"name":"Sarcoma epitelioide","definition":"Il sarcoma epitelioide è un tumore raro dei tessuti molli, caratterizzato da frequenti recidive locali, interessamento dei linfonodi e metastasi. Di solito colpisce i tessuti molli sottostanti la cute delle dita, delle mani, dell'avambraccio, della porzione inferiore della gamba o del piede; è raro l'interessamento di altre parti del corpo."},"nl":{"name":"Epithelioïd sarcoom","definition":"Epithelioïd sarcoom is een zeldzame tumor van weke delen die gekarakteriseerd wordt door een hoge incidentie van lokaal recidief, betrokkenheid van regionale lymfeklieren en metastasen op afstand. Het treft doorgaans de weke delen onder de huid van een vinger, hand, onderarm, onderbeen of voet, en minder vaak andere lichaamsstreken."},"pt":{"name":"Sarcoma epitelióide"},"pl":{"name":"Mięsak nabłonkopodobny","definition":"*Mięsak nabłonkowy jest rzadkim guzem tkanek miękkich, który charakteryzuje duża częstość miejscowych nawrotów, zajęcie regionalnych węzłów chłonnych i odległe przerzuty. Zwykle obejmuje tkanki miękkie pod skórą palca, dłoni, przedramienia, podudzia lub stopy, rzadziej inne obszary ciała."},"cs":{"name":"Epiteloidní sarkom"},"uk":{"name":"Епітеліоїдна саркома","definition":"A rare, soft tissue tumor characterized by high incidence of local recurrence, regional lymph node involvement and distant metastases. It commonly affects the soft tissue under the skin of a finger, hand, forearm, lower leg or foot, less often other areas of the body."},"zh":{"name":"上皮样肉瘤","definition":"上皮样肉瘤是一种罕见的软组织肿瘤，其特点是局部复发率高，局部淋巴结受累和远处转移。常累及手指、手、前臂、小腿或足部皮下软组织，很少累及身体其他部位。"},"tr":{"name":"Epiteloid sarkom"}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2B5F.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"932701232"},{"source":"ICD-11","code":"XH4F96","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1928606236"},{"source":"MONDO","code":"0017387","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0205944","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10181","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10015099","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":3394,"name":"Soft tissue sarcoma","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood"],"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 2011[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.04,"area":"United States","status":"Validated","source":"1922430[PMID]"}],"phenotypes":null}