{"orpha":458768,"name":"Papillary intralymphatic angioendothelioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Dabska tumor","PILA","Primary intralymphatic angioendothelioma"],"definition":"A rare vascular tumor characterized by an ill-defined, slowly growing, asymptomatic cutaneous plaque or nodule mostly involving the limbs, in fewer cases the trunk. The tumor is composed of lymphatic-like channels with prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. It is locally aggressive, while metastasis is rare. Infants and children are much more often affected than adults.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-458768/","orphanet_url":"https://www.orpha.net/en/disease/detail/458768","names":{"fr":{"name":"Angioendothéliome papillaire intralymphatique","synonyms":["Angioendothéliome intralymphatique primaire","Tumeur de Dabska"],"definition":"Tumeur vasculaire rare caractérisée par un nodule ou une plaque cutané(e) asymptomatique, aux contours mal définis et à croissance lente, se développant principalement sur les membres et, plus rarement, sur le tronc. La tumeur se compose de vaisseaux de type lymphatique en touffes papillaires intraluminales comportant des noyaux hyalins bordés par des cellules endothéliales en « clou de tapissier ». La tumeur est localement agressive, mais des métastases sont rarement observées. Les enfants en bas âge et les enfants plus âgés sont bien plus souvent touchés que les adultes."},"de":{"name":"Papilläres intralymphatisches Angioendotheliom","synonyms":["Dabska-Tumor","PILA"],"definition":"Ein seltener vaskulärer Tumor, der durch eine schlecht definierte, langsam wachsende, asymptomatische kutane Plaque oder ein Knötchen gekennzeichnet ist, das meist die Gliedmaßen, in selteneren Fällen auch den Rumpf betrifft. Der Tumor besteht aus lymphatisch-ähnlichen Kanälen mit ausgeprägten intraluminalen papillären Büscheln mit hyalinen Kernen, die von Hobnail-Endothelzellen ausgekleidet sind. Er ist lokal aggressiv, während Metastasen selten sind. Säuglinge und Kinder sind viel häufiger betroffen als Erwachsene."},"es":{"name":"Angioendotelioma intralinfático primario","synonyms":["Tumor de Dabska"],"definition":"Es un tumor vascular poco frecuente caracterizado por una placa o nódulo cutáneo asintomático, de crecimiento lento, mal definido, que afecta principalmente a las extremidades y en algunos casos al tronco. El tumor está compuesto por canales linfáticos con prominentes mechones papilares intraluminales con núcleos hialinos revestidos por células endoteliales en tachuela. Es localmente agresivo, aunque la metástasis es poco frecuente. Los lactantes y los niños se ven afectados con mucha mayor frecuencia que los adultos."},"it":{"name":"Angioendotelioma intralinfatico primitivo","definition":"Si tratta di un tumore vascolare raro, caratterizzato da una placca o da un nodulo cutaneo asintomatico, scarsamente definito, a crescita lenta, che si localizza per lo più sugli arti e, raramente, sul tronco. Il tumore è composto da vasi simil-linfatici con evidenti ciuffi papillari intraluminali con nuclei ialini rivestiti da cellule endoteliali ''a chiodo''. È localmente aggressivo, ma le metastasi sono rare. I neonati e i bambini sono molto più colpiti rispetto agli adulti."},"nl":{"name":"Primair intralymfatisch angio-endothelioom","synonyms":["Dabska-tumor"],"definition":"Een zeldzame vasculaire tumor, gekarakteriseerd door een onduidelijk gedefinieerde, traaggroeiende, asymptomatische cutane plaque of nodulus, doorgaans met betrokkenheid van ledematen, en in zeldzamere gevallen romp. De tumor bestaat uit lymfevat-achtige kanalen met prominente intraluminale papillaire tuften met hyaliene kernen, omzoomd door 'hobnail'-endotheelcellen. De tumor is lokaal agressief, maar metastase komt zelden voor. Zuigelingen en kinderen worden veel vaker getroffen dan volwassenen."},"pt":{"name":"Angioendotelioma intralinfático primário"},"pl":{"name":"Pierwotny wewnątrzlimfatyczny guz typu angioendotelioma","synonyms":["Guz Dąbskiej"]},"cs":{"name":"Primární intralymfatický angioendoteliom","synonyms":["Dabské tumor"]},"uk":{"name":"Папілярна інтралімфатична ангіоендотеліома","synonyms":["Пухлина Дабська","PILA","Первинна інтралімфатична ангіоендотеліома"],"definition":"A rare vascular tumor characterized by an ill-defined, slowly growing, asymptomatic cutaneous plaque or nodule mostly involving the limbs, in fewer cases the trunk. The tumor is composed of lymphatic-like channels with prominent intraluminal papillary tufts with hyaline cores lined by hobnail endothelial cells. It is locally aggressive, while metastasis is rare. Infants and children are much more often affected than adults."},"zh":{"name":"原发性淋巴管内血管内皮瘤","synonyms":["达布斯卡肿瘤"],"definition":"一种罕见的血管肿瘤，其特点是边界不清，生长缓慢，无症状的皮肤斑块或结节，多累及四肢，少数病例累及躯干。肿瘤由淋巴样管腔组成，管腔内乳头状突起伴透明核，排列有鞋钉样内皮细胞。本病局部侵袭性强，而转移罕见。婴幼儿和儿童比成人更易感。"},"tr":{"name":"Primer intralenfatik anjioendotelyoma","synonyms":["Dabska tümörü"]}},"xrefs":[{"source":"ICD-10","code":"D48.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018714","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346087","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","Childhood","Elderly","Infancy"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":30.0,"area":"Worldwide","status":"Validated","source":"21238627[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_21238627[PMID]"}],"phenotypes":null}