{"orpha":247762,"name":"Lipoblastoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare soft tissue tumor characterized by a lobulated, localized (lipoblastoma) or diffuse (lipoblastomatosis) lesion resembling fetal adipose tissue, composed of mature and immature adipocytes. It is most commonly found during the first years of life and presents as a slowly growing, well circumscribed mass, which may compress adjacent structures, depending on the location. Malignant transformation or metastasis does not occur, while recurrences are described especially in lipoblastomatosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-247762/","orphanet_url":"https://www.orpha.net/en/disease/detail/247762","names":{"fr":{"name":"Lipoblastome","definition":"Tumeur rare des tissus mous caractérisée par une tumeur lobulée, localisée (lipoblastome) ou diffuse (lipoblastomatose) semblable au tissu adipeux foetal, composée d'adipocytes matures et immatures. Elle se développe le plus souvent au cours des premières années de la vie et se présente sous la forme d'une masse à croissance lente, bien circonscrite, qui peut comprimer les structures adjacentes, en fonction de sa localisation. Il n'y a pas de transformation maligne ou de métastase, alors que des récidives sont décrites notamment dans la lipoblastomatose."},"de":{"name":"Lipoblastom","definition":"Ein seltener Weichteiltumor, der durch eine lobuläre, lokalisierte (Lipoblastom) oder diffuse (Lipoblastomatose) Läsion gekennzeichnet ist, die fötalem Fettgewebe ähnelt und aus reifen und unreifen Fettzellen besteht. Sie tritt am häufigsten in den ersten Lebensjahren auf und zeigt sich als langsam wachsende, gut umschriebene Gewebeverdichtung, die je nach Lokalisation auf benachbarte Strukturen drücken kann. Eine maligne Umwandlung oder Metastasierung findet nicht statt, während Rezidive insbesondere bei Lipoblastomatose beschrieben werden."},"es":{"name":"Lipoblastoma","definition":"Es un tumor de tejidos blandos poco frecuente caracterizado por una lesión lobulada, localizada (lipoblastoma) o difusa (lipoblastomatosis) similar al tejido adiposo fetal, compuesta por adipocitos maduros e inmaduros. Aparece con mayor frecuencia durante los primeros años de vida, presentándose como una masa de crecimiento lento bien delimitada, que puede comprimir las estructuras adyacentes, según la localización. No se ha descrito la transformación maligna ni metástasis, mientras que la recurrencia se describe principalmente en la lipoblastomatosis."},"it":{"name":"Lipoblastoma","definition":"Si tratta di un tumore raro dei tessuti molli, caratterizzato da una lesione lobulata localizzata (lipoblastoma) o diffusa (lipoblastomatosi) che ricorda il tessuto adiposo fetale, composta da adipociti maturi e immaturi. Di solito esordisce nei primi anni di vita sotto forma di massa ben delimitata a crescita lenta che, a seconda della localizzazione, può comprimere i tessuti adiacenti. Non si associa ad una evoluzione maligna o alle metastasi, ma sono stati descritte recidive, soprattutto della lipoblastomatosi."},"nl":{"name":"Lipoblastoom","definition":"Een zeldzame tumor van weke delen, gekarakteriseerd door een gelobde, gelokaliseerde (lipoblastoom) of diffuse (lipoblastomatose) laesie gelijkend op foetaal vetweefsel en bestaande uit rijpe en onrijpe adipocyten. De tumor wordt doorgaans ontdekt in de eerste levensjaren en presenteert zich als een traaggroeiende, goed begrensde massa die, afhankelijk van de locatie, druk kan uitoefenen op aangrenzende structuren. Maligne transformatie of metastase komt niet voor, terwijl recidiven vooral worden beschreven bij lipoblastomatose."},"pt":{"name":"Lipoblastoma"},"pl":{"name":"Lipoblastoma"},"cs":{"name":"Lipoblastom"},"uk":{"name":"Ліпобластома","definition":"A rare soft tissue tumor characterized by a lobulated, localized (lipoblastoma) or diffuse (lipoblastomatosis) lesion resembling fetal adipose tissue, composed of mature and immature adipocytes. It is most commonly found during the first years of life and presents as a slowly growing, well circumscribed mass, which may compress adjacent structures, depending on the location. Malignant transformation or metastasis does not occur, while recurrences are described especially in lipoblastomatosis."},"zh":{"name":"成脂细胞瘤","definition":"一种罕见的软组织肿瘤，其特征是分叶状、局部（成脂细胞瘤）或弥漫性（脂肪母细胞瘤病）病灶，类似胎儿脂肪组织，由成熟和未成熟的脂肪细胞组成。它最常见于生后最初几年，表现为一个缓慢生长的、边界良好的肿块，根据位置的不同，可能会压缩邻近的结构。不会发生恶化或转移，脂肪母细胞瘤病易复发。"},"tr":{"name":"Lipoblastom"}},"xrefs":[{"source":"ICD-10","code":"D17.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E80.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"581420938"},{"source":"MONDO","code":"0016611","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D062689","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1260965","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12015","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10074547","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":71209,"name":"Rare soft tissue tumor","classifications":[202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}