{"orpha":251663,"name":"Anaplastic oligoastrocytoma, dual genotype","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Anaplastic oligoastrocytoma, dual genotype NEC","Anaplastic oligoastrocytoma, dual genotype, not elsewhere classified","aMOA"],"definition":"A rare and aggressive glial tumor of the central nervous system, that usually presents in adults with seizures, is most often located in the cerebral hemispheres and that is associated with a very poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251663/","orphanet_url":"https://www.orpha.net/en/disease/detail/251663","names":{"fr":{"name":"Oligoastrocytome anaplasique, génotype hybride","synonyms":["aMOA","Oligoastrocytome anaplasique, génotype hybride NEC","Oligoastrocytome anaplasique, génotype hybride not elsewhere classified"],"definition":"L'oligoastrocytome anaplasique est une tumeur gliale rare et agressive du système nerveux central, qui se manifeste habituellement chez l'adulte par des convulsions. Il se trouve le plus souvent dans les hémisphères cérébraux et est associé à un très mauvais pronostic."},"de":{"name":"Anaplastisches Oligoastrozytom mit dualem Genotyp","synonyms":["Anaplastisches Oligoastrozytom mit doppeltem Genotyp, andernorts nicht klassifiziert","aMOA"],"definition":"Das anaplastische Oligoastrozytom ist ein seltener und aggressiver Glia-Tumor des zentralen Nervensystems, der sich bei Erwachsenen zumeist mit epileptischen Anfällen manifestiert. Er befindet sich am häufigsten in der zerebralen Hemisphäre und ist mit einer sehr schlechten Prognose verbunden."},"es":{"name":"Oligoastrocitoma anaplásico de genotipo dual","synonyms":["aMOA","Oligoastrocitoma anaplásico, genotipo dual, no clasificado en otra parte"],"definition":"Es un tumor glial poco frecuente y agresivo del sistema nervioso central, que suele presentarse en adultos con crisis epilépticas. La localización más frecuente es en los hemisferios cerebrales y está asociado con un pronóstico muy desfavorable."},"it":{"name":"Oligoastrocitoma anaplastico, genotipo ibrido","synonyms":["aMOA","Oligoastrocitoma anaplastico, genotipo ibrido NEC","Oligoastrocitoma anaplastico, genotipo ibrido, non classificato diversamente"],"definition":"L'oligoastrocitoma anaplastico è un tumore gliale, raro e aggressivo, del sistema nervoso centrale, che insorge di solito negli adulti con crisi epilettiche e si localizza più comunemente negli emisferi cerebrali, associandosi a una prognosi molto sfavorevole."},"nl":{"name":"Anaplastisch oligoastrocytoom, duaal genotype","synonyms":["aMOA","Anaplastisch oligoastrocytoom, duaal genotype NEC","Anaplastisch oligoastrocytoom, duaal genotype, niet elders geclassificeerd"],"definition":"Een zeldzame en agressieve gliale tumor van centraal zenuwstelsel die zich doorgaans presenteert bij volwassenen met insulten, meestal gesitueerd is in de cerebrale hemisferen, en geassocieerd is met een zeer slechte prognose."},"pt":{"name":"Oligoastrocitoma anaplásico","synonyms":["aMOA"]},"pl":{"name":"Skąpodrzewiakogwiaździak anaplastyczny","synonyms":["aMOA"]},"cs":{"name":"Anaplastický oligoastrocytom","synonyms":["aMOA"]},"uk":{"name":"Анапластична олігоастроцитома","synonyms":["aMOA"],"definition":"A rare and aggressive glial tumor of the central nervous system, that usually presents in adults with seizures, is most often located in the cerebral hemispheres and that is associated with a very poor prognosis."},"zh":{"name":"间变性少突星形细胞瘤","synonyms":["aMOA"],"definition":"一种罕见的侵袭性中枢神经系统胶质瘤，通常出现在有癫痫发作的成年人中，最常位于大脑半球，相关预后极差。"},"tr":{"name":"Anaplastik oligoastrositom","synonyms":["aMOA"]}},"xrefs":[{"source":"ICD-10","code":"C71.9","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2A00.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"185665409"},{"source":"MONDO","code":"0016703","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0431108","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10637","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251651,"name":"Oligoastrocytic tumor","classifications":[181,202]}],"children":[],"genes":[{"symbol":"IDH2","name":"isocitrate dehydrogenase (NADP(+)) 2","gene_type":"gene with protein product","association":"Biomarker tested in","status":"Assessed","source":"19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID]","hgnc":"5383","ensembl":"ENSG00000182054","omim":"147650","loci":["15q26.1"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[],"phenotypes":null}