{"orpha":58040,"name":"Osteoblastoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, neoplastic disease characterized by a typically benign, locally aggressive, non self-limiting, osteoblastic bone tumor, usually located on the spine, proximal humerus and hip (although any bone may be involved), generally manifesting with slowly progressive, dull aching pain which is difficult to localize and is not relieved by nonsteroidal anti-inflammatory drugs or aspirin. Neurologic symptoms, such as cranial nerve palsies, myelopathy, neuralgia, radiculopathy, paraparesis or paraplegia, may be associated if the spine is involved. Imaging reveals a lytic (or mixed lytic and blastic) lesion with a radiolucent nidus (> 2 cm) associated with reactive sclerotic bone.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-58040/","orphanet_url":"https://www.orpha.net/en/disease/detail/58040","names":{"fr":{"name":"Ostéoblastome","definition":"Maladie néoplasique rare caractérisée par une tumeur osseuse due à la prolifération des ostéoblastes, généralement bénigne, localement agressive et nécessitant un traitement. La maladie, dont la tumeur est habituellement localisée sur la colonne vertébrale, l'humérus proximal et la hanche (même si tout os peut être atteint) se manifeste généralement par une douleur sourde et persistante, à progression lente, qui est difficile à localiser et qui n'est pas soulagée par la prise d'anti-inflammatoires non stéroïdiens ou d'aspirine. Des symptômes neurologiques, tels qu'une paralysie du nerf crânien, une myélopathie, une névralgie, une radiculopathie, une paraparésie ou une paraplégie, peuvent être associés en cas d'atteinte de la colonne vertébrale. L'imagerie met en évidence une lésion lytique (ou une combinaison de lésions lytiques et blastiques) accompagnée d'un nidus radiotransparent (> 2 cm) associé à un os scléreux réactif."},"de":{"name":"Osteoblastom","definition":"Eine seltene Neoplasie, die durch einen typischerweise gutartigen, lokal aggressiven, nicht selbstlimitierenden, osteoblastischen Knochentumor gekennzeichnet ist, der in der Regel an der Wirbelsäule, dem proximalen Humerus und der Hüfte lokalisiert ist (obwohl jeder Knochen betroffen sein kann). Er manifestiert sich im Allgemeinen mit langsam fortschreitenden, dumpfen, schmerzenden Schmerzen, die schwer zu lokalisieren sind und durch nichtsteroidale entzündungshemmende Medikamente oder Aspirin nicht gelindert werden. Neurologische Symptome wie Hirnnervenlähmungen, Myelopathie, Neuralgie, Radikulopathie, Paraparese oder Querschnittslähmung können auftreten, wenn die Wirbelsäule betroffen ist. Die Bildgebung zeigt eine lytische (oder gemischt lytische und blastische) Läsion mit einem röntgendurchlässigen Nidus (> 2 cm) in Verbindung mit reaktivem sklerotischem Knochen."},"es":{"name":"Osteoblastoma","definition":"Es una enfermedad neoplásica poco frecuente caracterizada por un tumor óseo osteoblástico típicamente benigno, localmente agresivo, no autolimitado, localizado, por lo general, en la columna vertebral, el húmero proximal y la cadera (aunque puede afectar a cualquier hueso). Por lo habitual se manifiesta con dolor sordo lentamente progresivo de difícil localización que no remite al tratamiento con fármacos antiinflamatorios no esteroideos ni aspirina. En caso de afectación espinal, puede asociar síntomas neurológicos, tales como parálisis de los nervios craneales, mielopatía, neuralgia, radiculopatía o paraparesia. El estudio de imagen revela una lesión lítica, blástica o mixta con un nido radiolúcido (> 2 cm) asociado a hueso esclerótico reactivo."},"it":{"name":"Osteoblastoma","definition":"È una neoplasia rara caratterizzata da un tumore osseo osteoblastico di solito benigno, localmente aggressivo, non autolimitante, in genere localizzato sulla colonna, sulla porzione prossimale dell'omero e sull'anca (anche se può interessare tutte le ossa del corpo). Di solito esordisce con un dolore sordo che progredisce lentamente, di difficile localizzazione, che non si attenua con l'assunzione di farmaci antinfiammatori non steroidei o con l'aspirina. I pazienti con interessamento della colonna possono presentare sintomi neurologici, come le paralisi dei nervi cranici, la mielopatia, la nevralgia, la radicolopatia e la tetraparesi o la paraplegia. Le immagini diagnostiche evidenziano la presenza di una lesione litica (o mista litica e blastica) con un nido radiolucente (> 2 cm), associata ad osso sclerotico reattivo."},"nl":{"name":"Osteoblastoom","definition":"Een zeldzame, neoplastische ziekte die gekarakteriseerd wordt door een doorgaans goedaardige, lokaal agressieve, niet zelflimiterende, osteoblastische bottumor, meestal gesitueerd op de ruggengraat, proximale humerus en heup (hoewel eender welk bot kan aangetast zijn), en die zich doorgaans manifesteert met traag progressieve, doffe pijn die moeilijk te lokaliseren is en niet verzacht wordt door niet-steroïdale anti-inflammatoire geneesmiddelen of aspirine. Neurologische symptomen, zoals hersenzenuwverlammingen, myelopathie, neuralgie, radiculopathie, paraparese of paraplegie, kunnen geassocieerd zijn indien de ruggengraat betrokken is. Beeldvorming toont een lytisch (of gemengd lytisch en blastisch) letsel met een radiolucente nidus (> 2 cm) geassocieerd met reactief sclerotisch bot."},"pt":{"name":"Osteoblastoma"},"pl":{"name":"Osteoblastoma"},"cs":{"name":"Osteoblastom"},"uk":{"name":"Остеобластома","definition":"A rare, neoplastic disease characterized by a typically benign, locally aggressive, non self-limiting, osteoblastic bone tumor, usually located on the spine, proximal humerus and hip (although any bone may be involved), generally manifesting with slowly progressive, dull aching pain which is difficult to localize and is not relieved by nonsteroidal anti-inflammatory drugs or aspirin. Neurologic symptoms, such as cranial nerve palsies, myelopathy, neuralgia, radiculopathy, paraparesis or paraplegia, may be associated if the spine is involved. Imaging reveals a lytic (or mixed lytic and blastic) lesion with a radiolucent nidus (> 2 cm) associated with reactive sclerotic bone."},"zh":{"name":"成骨细胞瘤"},"tr":{"name":"Osteoblastom"}},"xrefs":[{"source":"ICD-10","code":"D16.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.4","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.5","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.6","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.7","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D16.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2E83.Z","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1948326341"},{"source":"MONDO","code":"0018936","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018215","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0029417","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":68411,"name":"Rare bone tumor","classifications":[199,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}