{"orpha":251957,"name":"Anaplastic ganglioglioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare mixed neuronal-glial tumor characterized by a mostly supratentorial space-occupying lesion often involving the temporal lobe, although it may occur anywhere in the central nervous system. The tumor shows anaplastic features in its glial component and is considered WHO grade III, which may, albeit inconsistently, indicate more aggressive behavior and less favorable prognosis. Clinical symptoms vary according to the location, the most common manifestation being seizures.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251957/","orphanet_url":"https://www.orpha.net/en/disease/detail/251957","names":{"fr":{"name":"Gangliogliome anaplasique","definition":"Tumeur mixte neuronale-gliale rare caractérisée par une lésion volumineuse généralement supratentorielle et affectant le plus souvent le lobe temporal, mais pouvant apparaitre dans toute partie du système nerveux central. Dans sa composante gliale, la tumeur se caractérise par une anaplasie et correspond au grade III de la classification de l'OMS, indiquant une forme plus agressive et un pronostic moins favorable de la maladie, bien qu'il s'agisse d'un trait inconstant. Les signes cliniques varient en fonction de la localisation, les symptômes les plus courants étant des crises convulsives."},"de":{"name":"Gangliogliom, anaplastisches","definition":"Ein seltener gemischter neuronal-glialer Tumor, der durch eine meist supratentorielle, raumfordernde Läsion gekennzeichnet ist, die häufig den Temporallappen betrifft, aber auch überall im Zentralnervensystem auftreten kann. Der Tumor weist in seiner glialen Komponente anaplastische Merkmale auf und wird als WHO-Grad III eingestuft, was, wenn auch nicht einheitlich, auf ein aggressiveres Verhalten und eine ungünstigere Prognose hinweisen kann. Die klinischen Symptome variieren je nach Lokalisation, am häufigsten treten Krampfanfälle auf."},"es":{"name":"Ganglioglioma anaplásico","definition":"Es un tumor mixto neuronal-glial poco frecuente caracterizado por una lesión ocupante de espacio principalmente supratentorial que a menudo afecta al lóbulo temporal, aunque puede aparecer en cualquier parte del sistema nervioso central. El tumor muestra hallazgos anaplásicos en su componente glial y está considerado como un tumor de grado III según la clasificación de la OMS, pudiendo indicar, aunque de manera inconsistente, un comportamiento más agresivo y un pronóstico menos favorable. Los síntomas clínicos varían según la localización, siendo las crisis epilépticas la manifestación más frecuente."},"it":{"name":"Ganglioglioma anaplastico","definition":"Si tratta di una neoplasia mista neuronale-gliale rara, caratterizzata da una lesione voluminosa prevalentemente sopratentoriale, che spesso interessa il lobo temporale, ma che può insorgere in qualsiasi area del sistema nervoso centrale. Il tumore presenta caratteristiche anaplastiche nella componente gliale, ed è classificato di grado III secondo i criteri dell'OMS; ciò può indicare un decorso più aggressivo e una prognosi meno favorevole, anche se si tratta di caratteristiche non costanti. I segni clinici più comuni sono le crisi epilettiche, ma i sintomi variano a seconda della localizzazione del tumore."},"nl":{"name":"Anaplastisch ganglioglioom","definition":"Een zeldzame gemengde neuronale-gliale tumor, gekarakteriseerd door een hoofdzakelijk supratentoriële, ruimte-innemende laesie die vaak temporaalkwab aantast, maar eender waar in centraal zenuwstelsel kan voorkomen. De tumor vertoont anaplastische kenmerken in de gliale component en wordt beschouwd als WHO-graad III, wat kan wijzen op agressiever gedrag en minder gunstige prognose, zij het niet consistent. Klinische symptomen variëren naargelang de locatie, en insulten zijn de meest gangbare manifestatie."},"pt":{"name":"Ganglioglioma anaplásico"},"pl":{"name":"Zwojakoglejak anaplastyczny"},"cs":{"name":"Anaplastický gangliogliom"},"uk":{"name":"Анапластична гангліогліома","definition":"A rare mixed neuronal-glial tumor characterized by a mostly supratentorial space-occupying lesion often involving the temporal lobe, although it may occur anywhere in the central nervous system. The tumor shows anaplastic features in its glial component and is considered WHO grade III, which may, albeit inconsistently, indicate more aggressive behavior and less favorable prognosis. Clinical symptoms vary according to the location, the most common manifestation being seizures."},"zh":{"name":"间变性神经节神经胶质瘤","definition":"一种罕见的神经元-胶质混合瘤，其特征是大部分位于幕上的占位性病变，通常累及颞叶，尽管它可能发生在中枢神经系统的任何地方。该肿瘤表现出胶质成分的间变性特征，被认为属于WHO III级，表明此病具有侵袭性进展和更差的预后，尽管并不都是如此。临床症状因部位不同而不同，最常见的表现是癫痫发作。"},"tr":{"name":"Anaplastik gangliogliom"}},"xrefs":[{"source":"ICD-10","code":"D43.0","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"2A00.21","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"813272495"},{"source":"ICD-11","code":"XH2GG3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1201112723"},{"source":"MONDO","code":"0016734","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0431112","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10639","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10089832","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]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}