{"orpha":480553,"name":"Aneurysmal bone cyst","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare bone tumor characterized by a benign, cystic lesion consisting of blood-filled cavities divided by fibrous septa containing fibroblasts, multinucleated osteoclast-type giant cells, and reactive woven bone. The tumor may arise <i>de novo</i> or secondarily, complicating other benign or malignant bone tumors. It most commonly arises during the first two decades of life and often affects the epiphyses of long bones and posterior elements of vertebral bodies. Patients typically present with pain and swelling, or neurological symptoms due to compression of nerve roots or the spinal cord by vertebral tumors.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-480553/","orphanet_url":"https://www.orpha.net/en/disease/detail/480553","names":{"fr":{"name":"Kyste osseux anévrysmal","synonyms":["KOA","Kyste anévrysmal de l'os"],"definition":"Tumeur osseuse rare caractérisée par une lésion kystique bénigne constituée de cavités remplies de sang et divisées par des septa fibreux contenant des fibroblastes, des cellules géantes multinucléées de type ostéoclastique et du tissu osseux « tissé ». La tumeur peut apparaître <i>de novo</i> ou en tant que complication dans l'évolution d'autres tumeurs osseuses bénignes ou malignes. Elle survient le plus souvent au cours des deux premières décennies de la vie et touche souvent les épiphyses des os longs et l'arc vertébral postérieur des corps vertébraux. Les patients présentent généralement des douleurs et des oedèmes, ou des symptômes neurologiques dus à la compression, des racines nerveuses ou de la moelle épinière par les tumeurs vertébrales."},"de":{"name":"Aneurysmatische Knochenzyste","definition":"Ein seltener Knochentumor, der durch eine gutartige zystische Läsion gekennzeichnet ist, die aus blutgefüllten Hohlräumen besteht, die durch fibröse Septen unterteilt sind und Fibroblasten, mehrkernige osteoklastenartige Riesenzellen und reaktives Knochengewebe enthalten. Der Tumor kann <i>de novo</i> oder sekundär als Komplikation anderer gutartiger oder bösartiger Knochentumoren entstehen. Er tritt meist in den ersten beiden Lebensjahrzehnten auf und befällt häufig die Epiphysen der Röhrenknochen und die hinteren Elemente der Wirbelkörper. Die Patienten präsentieren sich typischerweise mit Schmerzen und Schwellungen oder neurologischen Symptomen, die auf eine Kompression der Nervenwurzeln oder des Rückenmarks durch die Wirbeltumoren zurückzuführen sind."},"es":{"name":"Quiste óseo aneurismático","definition":"Es un tumor óseo poco frecuente caracterizado por una lesión quística, benigna compuesta por cavidades llenas de sangre divididas por tabiques fibrosos que contienen fibroblastos, células gigantes multinucleadas de tipo osteoclasto y tejido óseo reactivo. El tumor puede aparecer <i>de novo</i> o de manera secundaria, a partir de otros tumores óseos, benignos o malignos. Se origina con mayor frecuencia en las dos primeras décadas de vida, afectando a menudo a las epífisis de los huesos largos y a los elementos posteriores de los cuerpos vertebrales. Los pacientes suelen presentarse con dolor e inflamación o síntomas neurológicos debido a la compresión de las raíces nerviosas o la médula espinal por los tumores vertebrales."},"it":{"name":"Cisti ossea aneurismatica","definition":"Si tratta di un tumore osseo raro, caratterizzato da una lesione cistica benigna costituita da cavità contenenti sangue suddivisa da setti fibrosi contenenti fibroblasti, cellule giganti multinucleate di tipo osteoclastico e tessuto osseo reattivo a fibre intrecciate. Il tumore può insorgere <i>de novo</i> oppure come complicanza di altri tumori ossei benigni o maligni. Di solito esordisce nelle prime due decadi di vita e spesso interessa le epifisi delle ossa lunghe e la porzione posteriore dei corpi vertebrali. In genere i pazienti presentano dolore e tumefazione, o sintomi neurologici secondari alla compressione delle radici nervose o del midollo spinale da parte dei tumori vertebrali."},"nl":{"name":"Aneurysmatische botcyste","synonyms":["ABC"],"definition":"Een zeldzame tumor van bot, gekarakteriseerd door een goedaardige, cystische laesie bestaande uit met bloed gevulde holtes gescheiden door fibreuze septa die fibroblasten bevatten, multinucleaire osteoclast-type reuscellen, en reactief geweven bot. De tumor kan <i>de novo</i> ontstaan, of secundair als complicatie bij andere goed- of kwaadaardige bottumoren. De tumor ontstaat doorgaans tijdens de eerste twee levensdecennia, en treft vaak epifyses van pijpbeenderen en posterieure elementen van wervellichamen. Patiënten presenteren zich doorgaans met pijn en zwelling, of met neurologische symptomen als gevolg van compressie van zenuwwortels of van ruggenmerg door vertebrale tumoren."},"pt":{"name":"Quisto ósseo aneurismático"},"pl":{"name":"Torbiel tętniakowata kości"},"cs":{"name":"Aneuryzmatická kostní cysta"},"uk":{"name":"Аневризмальна кісткова кіста","definition":"A rare bone tumor characterized by a benign, cystic lesion consisting of blood-filled cavities divided by fibrous septa containing fibroblasts, multinucleated osteoclast-type giant cells, and reactive woven bone. The tumor may arise <i>de novo</i> or secondarily, complicating other benign or malignant bone tumors. It most commonly arises during the first two decades of life and often affects the epiphyses of long bones and posterior elements of vertebral bodies. Patients typically present with pain and swelling, or neurological symptoms due to compression of nerve roots or the spinal cord by vertebral tumors."},"zh":{"name":"动脉瘤性骨囊肿"},"tr":{"name":"Anevrizmal kemik kisti"}},"xrefs":[{"source":"ICD-10","code":"M85.5","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"FB80.6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1603788294"},{"source":"ICD-11","code":"XH23E0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"191920280"},{"source":"MONDO","code":"0018815","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D017824","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0152244","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10002362","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93419,"name":"Rare bone disease"},"parents":[{"orpha":68411,"name":"Rare bone tumor","classifications":[199,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}