{"orpha":263413,"name":"Angiosarcoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare vascular tumor characterized by a malignant space-occupying lesion composed of cells variably recapitulating features of normal endothelium. It mostly develops as a cutaneous tumor and is much less frequently located in the deep soft tissue. Clinical presentation is an enlarging mass, sometimes with symptoms like coagulopathy, anemia, persistent hematoma, or bruisability. Some tumors are associated with pre-existing conditions, e. g. Klippel-Trenaunay syndrome, Maffucci syndrome, or following radiation, among others. Older age, retroperitoneal location, large size, and high mitotic activity are predictors for poor outcome.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-263413/","orphanet_url":"https://www.orpha.net/en/disease/detail/263413","names":{"fr":{"name":"Angiosarcome","definition":"Tumeur vasculaire rare, caractérisée par une lésion tumorale expansive composée de cellules reproduisant de manière variable les caractéristiques de l'endothélium normal. Elle se développe le plus souvent sous la forme d'une tumeur cutanée et sa localisation dans les tissus mous profonds est beaucoup plus rare. Sur le plan clinique, elle se présente sous la forme d'une masse grossissante, parfois accompagnée de symptômes tels qu'une coagulopathie, une anémie, un hématome persistant ou des contusions. Certaines tumeurs sont associées, entre autres, à des maladies préexistantes, par exemple le syndrome de Klippel-Trenaunay, le syndrome de Maffucci ou font suite à des radiations. Un âge plus avancé, une localisation rétropéritonéale, une taille importante et une forte activité mitotique sont des facteurs prédictifs d'un diagnostic défavorable."},"de":{"name":"Angiosarkom","definition":"Ein seltener vaskulärer Tumor, der durch eine maligne, raumfordernde Läsion gekennzeichnet ist, die aus Zellen besteht, die in unterschiedlicher Weise Merkmale des normalen Endothels aufweisen. Er entwickelt sich meist als Hauttumor und ist sehr viel seltener im tiefen Weichteilgewebe zu finden. Klinisch zeigt sich der Tumor als sich vergrößernde Gewebeverdichtung, manchmal mit Symptomen wie Koagulopathie, Anämie, persistierenden Hämatomen oder Blutergüssen. Einige Tumoren treten in Verbindung mit vorbestehenden Erkrankungen auf, z. B. dem Klippel-Trenaunay-Syndrom, dem Maffucci-Syndrom oder nach Bestrahlung. Höheres Alter, retroperitoneale Lage, starke Ausdehnung und hohe mitotische Aktivität sind Prädiktoren für eine ungünstige Prognose."},"es":{"name":"Angiosarcoma","definition":"Es un tumor vascular poco frecuente caracterizado por una lesión maligna ocupante de espacio, compuesta por células que recapitulan de forma variable las características del endotelio normal. Se desarrolla principalmente como un tumor cutáneo, localizándose con mucha menor frecuencia en los tejidos blandos profundos. Clínicamente, se presenta con una masa expansiva, en ocasiones, con síntomas tales como coagulopatía, anemia y persistencia o propensión a hematomas. Algunos tumores están asociados a trastornos preexistentes, p. ej. el síndrome de Klippel-Trénaunay, el síndrome de Maffucci o la exposición a radiación, entre otros. Una edad avanzada, la localización retroperitoneal, un gran tamaño y una elevada actividad mitótica son factores pronósticos desfavorables."},"it":{"name":"Angiosarcoma","definition":"Si tratta di un tumore vascolare raro, caratterizzato da una lesione voluminosa maligna, composta da cellule che riproducono in modo variabile le caratteristiche delll'endotelio normale. In genere si manifesta sotto forma di tumore cutaneo e molto raramente si localizza nei tessuti molli profondi. Si presenta come massa che cresce, talvolta in associazione con una coagulopatia, con l'anemia, con gli ematomi persistenti o con la tendenza alle ecchimosi. Alcuni tumori insorgono nel quadro di altre malattie, come la sindrome di Klippel-Trenaunay e di Maffucci, oppure a seguito di radiazioni. L'età avanzata, la localizzazione retroperitoneale, le grandi dimensioni e un'elevata attività mitotica predicono una prognosi sfavorevole."},"nl":{"name":"Angiosarcoom","definition":"Een zeldzame vasculaire tumor, gekarakteriseerd door een maligne, ruimte-innemende laesie bestaande uit cellen die variabel kenmerken van normaal endotheel vertonen. Het ontwikkelt meestal als een cutane tumor en is veel minder vaak gesitueerd in diepe weke delen. De klinische presentatie is een groeiende massa, soms met symptomen als coagulopathie, anemie, persisterend hematoom, of vatbaarheid voor kneuzing. Sommige tumoren zijn geassocieerd met onder meer reeds aanwezige aandoeningen, e.g. syndroom van Klippel-Trenaunay of syndroom van Maffucci, of met eerdere bestraling. Oudere leeftijd, retroperitoneale locatie, grote omvang, en sterke mitotische activiteit zijn predictief voor een slechte uitkomst."},"pt":{"name":"Angiosarcoma"},"pl":{"name":"Naczyniakomięsak"},"cs":{"name":"Angiosarkom"},"uk":{"name":"Ангіосаркома","definition":"A rare vascular tumor characterized by a malignant space-occupying lesion composed of cells variably recapitulating features of normal endothelium. It mostly develops as a cutaneous tumor and is much less frequently located in the deep soft tissue. Clinical presentation is an enlarging mass, sometimes with symptoms like coagulopathy, anemia, persistent hematoma, or bruisability. Some tumors are associated with pre-existing conditions, e. g. Klippel-Trenaunay syndrome, Maffucci syndrome, or following radiation, among others. Older age, retroperitoneal location, large size, and high mitotic activity are predictors for poor outcome."},"zh":{"name":"血管肉瘤","definition":"一种罕见的血管瘤，其特征为恶性占位性病变，由不同的细胞组成，表现出正常内皮的多种特征。它主要发展为皮肤肿瘤，较少发生在深部软组织。临床表现为进行性增大的肿物，有时伴有凝血障碍、贫血、持续性血肿或瘀伤等症状。有些肿瘤与先前存在的疾病有关，如Klippel-Trenaunay综合征、Maffucci综合征或放疗后等。年龄大、腹膜后位置、体积大、核分裂活性高是预后不良的预测因素。"},"tr":{"name":"Anjiyosarkom"}},"xrefs":[{"source":"ICD-10","code":"C49.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2B56","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"423002561"},{"source":"MONDO","code":"0016982","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0018923","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10002476","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":673466,"name":"Malignant vascular tumor","classifications":[147,198,202]},{"orpha":3394,"name":"Soft tissue sarcoma","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"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 2011[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.02,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}