{"orpha":251627,"name":"Oligodendroglioma grade 2","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Oligodendroglioma"],"definition":"A rare glial tumor characterized by a highly cellular lesion that is diffusly infiltrating at the periphery and consists of evenly-spaced monomorphic cells with the oligodendroglial phenotype. It typically occurs in the supratentorial white matter. Histologically, the cells are uniformly round to oval with round nuclei, delicate chromatin and small nucleoli. Most patients present with seizures.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251627/","orphanet_url":"https://www.orpha.net/en/disease/detail/251627","names":{"fr":{"name":"Oligodendrogliome grade 2","synonyms":["Oligodendrogliome"],"definition":"Tumeur gliale rare caractérisée par une lésion hypercellulaire qui s'infiltre de manière diffuse en périphérie et qui se compose de cellules monomorphes de phénotype oligodendroglial, espacées de manière régulière. Elle siège généralement dans la substance blanche, à l'étage sus-tentoriel du cerveau. L'examen histologique met en évidence des cellules uniformes rondes ou ovales aux noyaux ronds à chromatine fine avec de petits nucléoles. Des convulsions sont observées chez la plupart des patients."},"de":{"name":"Oligodendrogliom Grad 2","synonyms":["Oligodendrogliom"],"definition":"Ein seltener glialer Tumor, der durch eine hochzelluläre Läsion gekennzeichnet ist, die in der Peripherie diffus infiltriert und aus gleichmäßig verteilten monomorphen Zellen mit oligodendroglialem Phänotyp besteht. Sie tritt typischerweise in der supratentoriellen weißen Substanz auf. Histologisch sind die Zellen einheitlich rund bis oval mit runden Kernen, feinem Chromatin und kleinen Nukleoli. Die meisten Patienten präsentieren sich mit Krampfanfällen."},"es":{"name":"Oligodendroglioma de grado 2","synonyms":["Oligodendroglioma"],"definition":"Es un tumor glial poco frecuente caracterizado por una lesión hipercelular con infiltración difusa periférica consistente en células monomorfas con fenotipo oligodendroglial distribuidas uniformemente. Suele originarse en la sustancia blanca supratentorial. Histológicamente, las células son uniformemente redondas u ovaladas con núcleos redondos, cromatina fina y nucleolos pequeños. La mayoría de pacientes se presenta con crisis epilépticas."},"it":{"name":"Oligodendroglioma","definition":"Si tratta di una neoplasia gliale rara, caratterizzata da una lesione ipercellulare diffusamente infiltrante nell'area periferica, composta da cellule monomorfe distanziate in modo uniforme, con fenotipo oligodendrogliale. Di solito si localizza nella sostanza bianca della regione sopratentoriale. Dal punto di vista istologico, le cellule sono uniformemente tonde o ovali, con nuclei arrotondati, cromatina delicata e nucleoli di piccole dimensioni. La maggior parte dei pazienti presenta crisi epilettiche."},"nl":{"name":"Oligodendroglioom graad 2","synonyms":["Oligodendroglioom"],"definition":"Een zeldzame gliale tumor gekarakteriseerd door een zeer cellulair letsel dat diffuus infiltreert aan de periferie en bestaat uit gelijkmatig verspreide monomorfe cellen met een oligodendrogliaal fenotype. De tumor komt doorgaans voor in de supratentoriële witte stof. Histologisch zijn de cellen uniform rond tot ovaal met ronde kernen, delicaat chromatine en kleine kernlichaampjes. De meeste patiënten vertonen insulten."},"pt":{"name":"Oligodendroglioma"},"pl":{"name":"Skąpodrzewiak (Oligodendroglioma)","synonyms":["Oligodendroglioma"]},"cs":{"name":"Oligodendrogliom","synonyms":["Oligodendroglioma"]},"uk":{"name":"Олігодендрогліома","definition":"A rare glial tumor characterized by a highly cellular lesion that is diffusly infiltrating at the periphery and consists of evenly-spaced monomorphic cells with the oligodendroglial phenotype. It typically occurs in the supratentorial white matter. Histologically, the cells are uniformly round to oval with round nuclei, delicate chromatin and small nucleoli. Most patients present with seizures."},"zh":{"name":"少突胶质细胞瘤","definition":"一种罕见的胶质瘤，其特征是周边弥漫浸润的高度细胞性病变，由均匀分布的具有少突胶质表型的单形细胞组成。它通常发生在幕上白质。组织学上，细胞均匀圆形至椭圆形，细胞核圆形，染色质细腻，核仁细小。大多数患者都有癫痫发作的症状。"},"tr":{"name":"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":"805811519"},{"source":"ICD-11","code":"XH7K31","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1993147011"},{"source":"ICD-11","code":"XH7W59","qualifier":"BTNT","icd_relation":"specific","validated":true,"icd11_uri":"1235392779"},{"source":"MONDO","code":"0016695","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D009837","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0028945","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9953","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10030286","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":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.25,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}