{"orpha":458758,"name":"Composite hemangioendothelioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Polymorphous hemangioendothelioma"],"definition":"A rare vascular tumor characterized by a poorly circumscribed, infiltrative nodular lesion with vascular differentiation, centered in the dermis and subcutis. The tumor is composed of histologically benign, intermediate, and malignant components. Typical is an admixture of different components which include epithelioid and retiform hemangioendothelioma, spindle cell hemangioma, angiosarcoma-like areas, and benign vascular lesions. Predilection sites are the distal extremities. Many patients have a history of lymphedema. Local recurrence is frequent, while metastasis is rare.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-458758/","orphanet_url":"https://www.orpha.net/en/disease/detail/458758","names":{"fr":{"name":"Hémangioendothéliome composite","definition":"Tumeur vasculaire rare caractérisée par une lésion nodulaire peu circonscrite, infiltrée, avec une différenciation vasculaire, localisée dans le derme et le tissus sous-cutané. Sur le plan histologique, la tumeur est composée de tissus bénins, malins et à malignité intermédiaire. Il s'agit typiquement d'un mélange de différents tissus, à savoir, l'hémangioendothéliome épithélioïde et rétiforme, l'hémangiome à cellules fusiformes, des zones de type angiosarcome et des lésions vasculaires bénignes. Les sites de prédilection sont les muscles distaux des membres. De nombreux patients présentent des antécédents de lymphoedème. La récidive locale est fréquente, tandis que les métastases sont rares."},"de":{"name":"Hämangioendotheliom, zusammengesetztes","synonyms":["Polymorphes Hämangioendotheliom"],"definition":"Ein seltener vaskulärer Tumor, der durch eine schlecht umschriebene, infiltrierende, knotige Läsion mit vaskulärer Differenzierung gekennzeichnet ist, die in der Dermis und Subkutis zentriert ist. Der Tumor setzt sich histologisch aus gutartigen, intermediären und malignen Komponenten zusammen. Typisch ist eine Vermischung verschiedener Komponenten, zu denen epitheloide und retiforme Hämangioendotheliome, spindelzellige Hämangiome, angiosarkomähnliche Areale und gutartige Gefäßläsionen gehören. Prädilektionsstellen sind die distalen Extremitäten. Viele Patienten haben in der Vorgeschichte ein Lymphödem. Lokalrezidive sind häufig, Metastasen dagegen selten."},"es":{"name":"Hemangioendotelioma compuesto","synonyms":["Hemangioendotelioma polimorfo"],"definition":"Es un tumor vascular poco frecuente caracterizado por una lesión nodular, infiltrativa, mal delimitada con diferenciación vascular, localizada en la dermis y el subcutis. El tumor está compuesto por elementos histológicamente benignos, intermedios y malignos. Típicamente es una mezcla de componentes diferentes, que incluyen el hemangioendotelioma epitelioide y retiforme, el hemangioma de células fusiformes, áreas similares al angiosarcoma y lesiones vasculares benignas. Muestra predilección por la parte distal de las extremidades. Muchos pacientes presentan antecedentes de linfedema. Las recidivas locales son frecuentes, mientras que la metástasis es poco común."},"it":{"name":"Emangioendotelioma composito","synonyms":["Emangioendotelioma polimorfo"],"definition":"Si tratta di un tumore vascolare raro caratterizzato da una lesione nodulare infiltrante scarsamente circoscritta con differenziazione vascolare, localizzata nel derma e nella regione sottocutanea. Dal punto di vista istologico, il tumore è composto da componenti benigne, intermedie e maligne. È comune la commistione tra diverse componenti, che comprendono l'emangioendotelioma epitelioide e retiforme, l'emangioma a cellule fusiformi, le zone simil-angiosarcoma e le lesioni vascolari benigne. Si localizza prevalentemente nella porzione distale degli arti. Molti pazienti hanno un'anamnesi positiva per il linfedema. Le recidive locali sono comuni, mentre le metastasi sono rare."},"nl":{"name":"Samengesteld hemangio-endothelioom","synonyms":["Composiet hemangio-endothelioom","Polymorf hemangio-endothelioom"],"definition":"Een zeldzame vasculaire tumor, gekarakteriseerd door een weinig begrensde, infiltratieve nodulaire laesie met vasculaire differentiatie, gecentreerd in dermis en subcutis. De tumor bestaat uit histologisch goedaardige, intermediaire, en maligne componenten. Typerend is een mengeling van verschillende componenten, waaronder epithelioïd en retiform hemangio-endothelioom, spoelcelhemangioom, angiosarcoom-achtige gebieden, en goedaardige vasculaire laesies. Distale ledematen zijn het vaakst aangetast. Vele patiënten hebben een geschiedenis van lymfoedeem. Lokaal recidief komt vaak voor, terwijl metastase zeldzaam is."},"pt":{"name":"Hemangioendotelioma composto"},"pl":{"name":"Naczyniakośródbłoniak złożony","synonyms":["Polymorphous hemangioendothelioma"]},"cs":{"name":"Složený hemangioendoteliom","synonyms":["Polymorphous hemangioendothelioma"]},"uk":{"name":"Композитна гемангіоендотеліома","definition":"A rare vascular tumor characterized by a poorly circumscribed, infiltrative nodular lesion with vascular differentiation, centered in the dermis and subcutis. The tumor is composed of histologically benign, intermediate, and malignant components. Typical is an admixture of different components which include epithelioid and retiform hemangioendothelioma, spindle cell hemangioma, angiosarcoma-like areas, and benign vascular lesions. Predilection sites are the distal extremities. Many patients have a history of lymphedema. Local recurrence is frequent, while metastasis is rare."},"zh":{"name":"复合性血管内皮瘤","definition":"是一种罕见的血管性肿瘤，其特点是以真皮和皮下组织为中心的界限不清、浸润性结节性病变伴血管分化。肿瘤由组织学上的良性、中度和恶性成分组成。不同的混合病变较典型，其中包括上皮样和网状血管样内皮瘤、梭形细胞血管瘤、血管肉瘤样区域和良性血管病变。好发部位为四肢远端。许多患者有淋巴水肿史。局部复发频繁，而转移罕见。"},"tr":{"name":"Kompozit hemanjioendotelyoma"}},"xrefs":[{"source":"ICD-10","code":"D48.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018712","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1304513","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":673473,"name":"Borderline vascular tumor","classifications":[147,198,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":39.0,"area":"Worldwide","status":"Validated","source":"25666464[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25666464[PMID]"}],"phenotypes":null}