{"orpha":251949,"name":"Ganglioglioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Ganglioglioma is a rare, usually benign, well-circumscribed, often cystic, mixed neuronal-glial tumor (composed of both neoplastic glial and ganglionic elements) which is typically located in the temporal lobe and rarely invades the surrounding tissue. Patients usually present with seizures refractory to medical treatment. Association with neurofibromatosis type I and tuberous sclerosis has been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251949/","orphanet_url":"https://www.orpha.net/en/disease/detail/251949","names":{"fr":{"name":"Gangliogliome","definition":"Le gangliogliome est une tumeur mixte neuronale et gliale rare (composée à la fois d'éléments néoplasiques gliaux et ganglionnaires), bien circonscrite, souvent kystique et généralement bénigne. Elle se trouve généralement dans le lobe temporal et envahit rarement le tissu environnant. Les patients présentent généralement des convulsions réfractaires aux traitements médicaux. Une association avec la neurofibromatose type 1 et la sclérose tubéreuse a été rapportée."},"de":{"name":"Gangliogliom","definition":"Das Gangliogliom ist ein seltener, meist gutartiger, gut umschriebener, oft zystischer, gemischter neuronal-glialer Tumor (bestehend sowohl aus neoplastischen glialen als auch ganglionären Elementen), der typischerweise im Schläfenlappen lokalisiert ist und selten in das umliegende Gewebe eindringt. Die Patienten präsentieren sich in der Regel mit Anfällen, die auf eine medikamentöse Behandlung refraktär sind. Es wurde über eine Assoziation mit Neurofibromatose Typ I und tuberöser Sklerose berichtet."},"es":{"name":"Ganglioglioma","definition":"El ganglioglioma es un tumor mixto neuronal-glial (compuesto por elementos neoplásicos tanto gliales como gangliónicos) poco frecuente, por lo general benigno, bien delimitado y a menudo quístico, típicamente localizado en el lóbulo temporal y que rara vez invade el tejido circundante. Por lo habitual, los afectados se presentan con crisis refractarias al tratamiento médico. Se ha descrito la asociación con la neurofibromatosis tipo I y la esclerosis tuberosa."},"it":{"name":"Ganglioglioma","definition":"Il ganglioglioma è un tumore raro, misto neuronale-gliale (composto da elementi neoplastici gliali e ganglionici), di solito benigno, ben circoscritto, spesso di natura cistica. Per lo più si localizza nel lobo temporale e solo raramente invade i tessuti circostanti. In genere i pazienti presentano crisi epilettiche refrattarie al trattamento. Può associarsi alla neurofibromatosi tipo I e alla sclerosi tuberosa."},"nl":{"name":"Ganglioglioom","definition":"Ganglioglioom is een zeldzame, meestal goedaardige, goed begrensde, vaak cystische, gemengde neuronale-gliale tumor (bestaande uit zowel neoplastische gliale en ganglion elementen) die doorgaans voorkomt in de temporale kwab en zelden omringend weefsel binnendringt. Patiënten vertonen meestal insulten die ongevoelig zijn voor medische behandeling. Er werd ook associatie met neurofibromatose type I en tubereuze sclerose gerapporteerd."},"pt":{"name":"Ganglioglioma"},"pl":{"name":"Zwojakoglejak"},"cs":{"name":"Gangliogliom"},"uk":{"name":"Гангліогліома","definition":"Ganglioglioma is a rare, usually benign, well-circumscribed, often cystic, mixed neuronal-glial tumor (composed of both neoplastic glial and ganglionic elements) which is typically located in the temporal lobe and rarely invades the surrounding tissue. Patients usually present with seizures refractory to medical treatment. Association with neurofibromatosis type I and tuberous sclerosis has been reported."},"zh":{"name":"神经节神经胶质瘤","definition":"神经节细胞胶质瘤是一种罕见的混合性神经元-神经胶质瘤(由肿瘤性胶质细胞和神经节细胞组成)，通常是良性的，边界清楚，主要是囊性的，通常位于颞叶，很少侵犯周围组织。患者通常会出现难治性癫痫发作。也有与神经纤维瘤病I型和结节性硬化症相关的报道。"},"tr":{"name":"Gangliogliom"}},"xrefs":[{"source":"ICD-10","code":"D33.0","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"2A00.21","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1287417975"},{"source":"MONDO","code":"0016733","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018303","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206716","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"2430","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10017701","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251934,"name":"Mixed neuronal-glial tumor","classifications":[181,202]},{"orpha":101998,"name":"Rare epilepsy","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}