{"orpha":251630,"name":"Oligodendroglioma grade 3","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Anaplastic oligodendroglioma"],"definition":"A rare glial tumor characterized by a grade III oligodendroglial tumour with focal or diffuse anaplastic features. It typically occurs in the supratentorial white matter. Histologically, the cells are enlarged and epithelioid with pleomorphic and increased size nuclei, a vesicular chromatin pattern and prominent nucleoli. Most patients present with seizures.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251630/","orphanet_url":"https://www.orpha.net/en/disease/detail/251630","names":{"fr":{"name":"Oligodendrogliome grade 3","synonyms":["Oligodendrogliome anaplasique"],"definition":"Tumeur gliale rare caractérisée par une tumeur oligodendrogliale de grade III dotée de caractéristiques anaplasiques focales ou diffuses. Elle siège généralement dans la substance blanche, à l'étage sus-tentoriel du cerveau. L'examen histologique révèle des cellules volumineuses et épithélioïdes avec de gros noyaux pléomorphes, à chromatine vésiculaire et dotés de nucléoles proéminentes. Des convulsions sont observées chez la plupart des patients."},"de":{"name":"Oligodendrogliom Grad 3","synonyms":["Oligodendrogliom, anaplastisches"],"definition":"Ein seltener Tumor aus der Gruppe der Gliome, der als oligodendroglialen Tumor des WHO Grades III definiert ist und durch fokale oder diffuse anaplastische Merkmalen gekennzeichnet ist. Er tritt typischerweise in der supratentoriellen weißen Substanz auf. Histologisch sind die Zellen vergrößert und epitheloid mit pleomorphen und vergrößerten Zellkernen, einem vesikulären Chromatinmuster und prominenten Nukleoli. Die meisten Patienten präsentieren sich mit Krampfanfällen."},"es":{"name":"Oligodendroglioma de grado 3","synonyms":["Oligodendroglioma anaplásico"],"definition":"Es una neoplasia glial poco frecuente caracterizada por un tumor oligodendroglial de grado III con hallazgos anaplásicos focales o difusos. Aparece típicamente en la sustancia blanca supratentorial. Histológicamente, se compone de células grandes y epitelioides con núcleos agrandados y pleomórficos, cromatina vesicular y nucleolos prominentes. La mayoría de los pacientes se presenta con crisis epilépticas."},"it":{"name":"Oligodendroglioma anaplastico","definition":"Si tratta di una neoplasia gliale rara che consiste in un tumore oligodendrogliale con caratteristiche anaplastiche focali o diffuse. Di solito si localizza nella sostanza bianca della regione sopratentoriale. L'esame istologico evidenzia cellule ingrandite ed epitelioidi, con nuclei pleomorfi di dimensioni superiori alla norma, cromatina dall'aspetto vescicolare e nucleoli prominenti. La maggior parte dei pazienti presenta crisi epilettiche."},"nl":{"name":"Oligodendroglioom graad 3","synonyms":["Anaplastisch oligodendroglioom"],"definition":"Een zeldzame gliale tumor gekarakteriseerd door een graad III oligodendrogliale tumor met focale of diffuse anaplastische kenmerken. De tumor komt doorgaans voor in de supratentoriële witte stof. Histologisch zijn de cellen vergroot en epithelioïd met pleomorfe en vergrote kernen, een vesiculair chromatinepatroon en prominente nucleoli. De meeste patiënten vertonen insulten."},"pt":{"name":"Oligodendroglioma anaplásico"},"pl":{"name":"Skapodrzewiak anaplastyczny","synonyms":["Anaplastic oligodendroglioma"]},"cs":{"name":"Anaplastický oligodendrogliom","synonyms":["Anaplastic oligodendroglioma"]},"uk":{"name":"Анапластична олігодендрогліома","definition":"A rare glial tumor characterized by a grade III oligodendroglial tumour with focal or diffuse anaplastic features. It typically occurs in the supratentorial white matter. Histologically, the cells are enlarged and epithelioid with pleomorphic and increased size nuclei, a vesicular chromatin pattern and prominent nucleoli. Most patients present with seizures."},"zh":{"name":"间变性少突胶质细胞瘤","definition":"一种罕见的胶质瘤，其特征为三级少突胶质细胞瘤，伴有局灶性或弥漫性间变性特征。它通常发生在幕上白质。组织学上，细胞增大呈上皮样，伴有多形且增大的细胞核，染色质呈泡状，核仁突出。大多数患者都有癫痫发作的症状。"},"tr":{"name":"Anaplastik oligodendrogliom"}},"xrefs":[{"source":"OMIM","code":"137800","qualifier":"NTBT","icd_relation":null,"validated":true},{"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":"774024406"},{"source":"ICD-11","code":"XH9QF3","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1384769153"},{"source":"MONDO","code":"0016696","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334590","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9472","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10026659","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":46484,"name":"Oligodendroglial 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":"23681562[PMID]_19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID]","hgnc":"5383","ensembl":"ENSG00000182054","omim":"147650","loci":["15q26.1"]},{"symbol":"POT1","name":"protection of telomeres 1","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"25482530[PMID]","hgnc":"17284","ensembl":"ENSG00000128513","omim":"606478","loci":["7q31.33"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.09,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.11,"area":"United States","status":"Validated","source":"Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID]"}],"phenotypes":null}