{"orpha":458763,"name":"Retiform hemangioendothelioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare vascular tumor characterized by a slowly growing lesion with predominant involvement of the skin and subcutaneous tissue of the distal extremities. Distinctive arborizing blood vessels lined by endothelial cells with characteristic hobnail morphology are a typical feature. Local recurrences are frequent unless wide local excision is performed, while metastasis is rare.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-458763/","orphanet_url":"https://www.orpha.net/en/disease/detail/458763","names":{"fr":{"name":"Hémangioendothéliome rétiforme","definition":"Tumeur vasculaire rare caractérisée par une tumeur à croissance lente avec une atteinte prédominante de la peau et du tissu sous-cutané de la partie distale des membres. Elle se caractérise par des vaisseaux sanguins disposés en arborescence, bordés de cellules endothéliales présentant un aspect clouté. Les récidives locales sont fréquentes, sauf en cas de résection qui doit être la plus large possible, tandis que les métastases sont rares."},"de":{"name":"Retiformes Hämangioendotheliom","definition":"Ein seltener vaskulärer Tumor, der durch eine langsam wachsende Läsion gekennzeichnet ist, die vorwiegend die Haut und das subkutane Gewebe der distalen Extremitäten befällt. Ein typisches Merkmal sind ausgeprägte, verzweigte Blutgefäße, die von Endothelzellen mit charakteristischer Hobnail-Morphologie ausgekleidet sind. Lokalrezidive sind häufig, es sei denn, es wird eine breite lokale Exzision durchgeführt, während Metastasen selten sind."},"es":{"name":"Hemangioendotelioma retiforme","definition":"Es un tumor vascular poco frecuente caracterizado por una lesión de crecimiento lento con una afectación predominantemente cutánea y subcutánea en la parte distal de las extremidades. Una característica típica es la presencia de vasos sanguíneos arborescentes, revestidos por células endoteliales con una característica morfología en tachuela. Las recidivas locales son frecuentes a menos que se realice una escisión local amplia, aunque que las metástasis son poco habituales."},"it":{"name":"Emangioendotelioma retiforme","definition":"Si tratta di una neoplasia vascolare rara, caratterizzata da una lesione a crescita lenta che interessa per lo più la cute e il tessuto sottocutaneo nella porzione distale degli arti. Si distingue per la presenza di vasi sanguigni dall'aspetto arborescente rivestiti da cellule endoteliali con morfologia ''a chiodo''. Sono frequenti le recidive locali, a meno che non venga eseguita un'ampia resezione locale. Le metastasi sono rare."},"nl":{"name":"Retiform hemangio-endothelioom","definition":"Een zeldzame vasculaire tumor, gekarakteriseerd door een traaggroeiende laesie met voornamelijk betrokkenheid van huid en onderhuids weefsel van distale ledematen. Kenmerkende sterk vertakte bloedvaten bekleed met endotheelcellen met karakteristieke kopspijker-morfologie zijn een typisch kenmerk. Locale recidiven komen vaak voor, tenzij ruime locale excisie wordt uitgevoerd, terwijl metastase zeldzaam is."},"pt":{"name":"Hemangioendotelioma retiforme"},"pl":{"name":"Naczyniakośródbłoniak sieciowaty"},"cs":{"name":"Retiformní hemangioendoteliom"},"uk":{"name":"Ретиформна гемангіоендотеліома","definition":"A rare vascular tumor characterized by a slowly growing lesion with predominant involvement of the skin and subcutaneous tissue of the distal extremities. Distinctive arborizing blood vessels lined by endothelial cells with characteristic hobnail morphology are a typical feature. Local recurrences are frequent unless wide local excision is performed, while metastasis is rare."},"zh":{"name":"网状血管内皮瘤","definition":"一种罕见的血管肿瘤，其特点是病变生长缓慢，主要累及四肢远端皮肤和皮下组织。其典型的特征是独特树状血管，内部排列着特征性的鞋钉样形态的内皮细胞。除非进行广泛的局部切除，否则常有局部复发，而转移则罕见。"},"tr":{"name":"Retiform hemanjioendotelyoma"}},"xrefs":[{"source":"ICD-10","code":"D48.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018713","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1304512","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":["Adolescent","Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":32.0,"area":"Worldwide","status":"Validated","source":"25812740[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_25812740[PMID]"}],"phenotypes":null}