{"orpha":251595,"name":"Astrocytoma grade 2","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Diffuse astrocytoma"],"definition":"A rare low-grade astrocytoma characterized by a high degree of cellular differentiation, slow growth, and diffuse infiltration of adjacent brain structures, and corresponding to WHO grade II. The tumor typically affects young adults and has an intrinsic tendency for progression to high-grade glioma. Histological variants are fibrillary, gemistocytic, and protoplasmic astrocytoma. Patients most commonly present with seizures, but also with other neurological or neuropsychological abnormalities, depending on the location.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251595/","orphanet_url":"https://www.orpha.net/en/disease/detail/251595","names":{"fr":{"name":"Astrocytome de grade 2","synonyms":["Astrocytome diffus"],"definition":"Astrocytome rare de bas grade caractérisé par un haut degré de différenciation cellulaire, une croissance lente et une infiltration diffuse des tissus cérébraux adjacents, correspondant au grade II de la classification de l'OMS. La tumeur touche généralement les jeunes adultes et a tendance à évoluer vers un gliome de haut grade. Les variantes histologiques sont l'astrocytome fibrillaire, gémistocytique et protoplasmique. Selon la localisation de la tumeur, les patients présentent le plus souvent des crises convulsives, mais aussi d'autres anomalies neurologiques ou neuropsychologiques."},"de":{"name":"Astrozytom Grad 2","synonyms":["Astrozytom, diffuses"],"definition":"Ein niedriggradiges Astrozytom, das sich durch einen hohen Grad an zellulärer Differenzierung, langsames Wachstum und eine diffuse Infiltration benachbarter Hirnstrukturen auszeichnet und dem WHO-Grad II entspricht. Der Tumor betrifft in der Regel junge Erwachsene und hat eine intrinsische Tendenz zur Progression zu einem hochgradigen Gliom. Histologische Varianten sind fibrilläre, gemistozytäre und protoplasmatische Astrozytome. Am häufigsten treten bei den Patienten Krampfanfälle auf, aber je nach Lokalisation auch andere neurologische oder neuropsychologische Auffälligkeiten."},"es":{"name":"Astrocitoma de grado 2","synonyms":["Astrocitoma difuso"],"definition":"Es un astrocitoma poco frecuente, de bajo grado, caracterizado por un alto grado de diferenciación celular, crecimiento lento, e infiltración difusa en las estructuras cerebrales adyacentes, se corresponde con el grado II de la clasificación de la OMS. Por lo general, el tumor afecta a adultos jóvenes y posee una tendencia intrínseca a progresar a un glioma de alto grado. Las variantes histológicas son el astrocitoma fibrilar, el gemistocítico y el protoplásmico. Los pacientes se presentan, con mayor frecuencia, con crisis epilépticas, aunque también con otras anomalías neurológicas o neuropsicológicas, según la localización."},"it":{"name":"Astrocitoma di grado 2","synonyms":["Astrocitoma diffuso"],"definition":"Si tratta di un astrocitoma raro di basso grado, caratterizzato da un elevato grado di differenziazione cellulare, crescita lenta e infiltrazione diffusa delle strutture cerebrali adiacenti, corrispondente al grado II della classificazione dell'OMS. Di solito colpisce i giovani adulti e tende a progredire verso il glioma di grado elevato. Le varianti istologiche comprendono l'astrocitoma fibrillare, gemistocitico e protoplasmatico. In genere i pazienti presentano crisi epilettiche, ma anche altre anomalie neurologiche o neuropsicologiche, a seconda della localizzazione del tumore."},"nl":{"name":"Astrocytoom graad 2","synonyms":["Diffuus astrocytoom"],"definition":"Een zeldzaam, laaggradig astrocytoom, gekarakteriseerd door een hoge mate van cellulaire differentiatie, trage groei, en diffuse infiltratie van aangrenzende structuren van de hersenen, en geclassificeerd als WHO-graad II. De tumor treft doorgaans jonge volwassenen en heeft een intrinsieke neiging tot progressie naar hooggradig glioom. Histologische varianten zijn fibrillair, gemistocytair, en protoplasmatisch astrocytoom. Patiënten presenteren zich meestal met insulten, maar ook met andere neurologische of neuropsychologische afwijkingen, afhankelijk van de locatie."},"pt":{"name":"Astrocitoma difuso"},"pl":{"name":"Gwiaździak rozlany","synonyms":["Diffuse astrocytoma"]},"cs":{"name":"Difuzní astrocytom","synonyms":["Diffuse astrocytoma"]},"uk":{"name":"Дифузна астроцитома","definition":"A rare low-grade astrocytoma characterized by a high degree of cellular differentiation, slow growth, and diffuse infiltration of adjacent brain structures, and corresponding to WHO grade II. The tumor typically affects young adults and has an intrinsic tendency for progression to high-grade glioma. Histological variants are fibrillary, gemistocytic, and protoplasmic astrocytoma. Patients most commonly present with seizures, but also with other neurological or neuropsychological abnormalities, depending on the location."},"zh":{"name":"弥漫性星形细胞瘤","definition":"一种罕见的低级别星形细胞瘤，其特征是细胞分化程度高，生长缓慢，弥漫性浸润邻近的脑结构，属于WHO II级。该肿瘤主要在青年人群中发生，并有向高级别胶质瘤发展的内在倾向。组织学变异有纤维性星形细胞瘤、双核细胞星形细胞瘤和原浆星形细胞瘤。患者最常见的症状是癫痫发作，但也有其他神经或神经心理异常，具体取决于病灶的位置。"},"tr":{"name":"Yaygın astrositom"}},"xrefs":[{"source":"ICD-10","code":"D33.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A00.0Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"788642151"},{"source":"MONDO","code":"0016686","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0280785","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5907","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251592,"name":"Low-grade astrocytoma","classifications":[181,202]}],"children":[{"orpha":251601,"name":"Fibrillary astrocytoma","type":"Histopathological subtype"},{"orpha":251604,"name":"Gemistocytic astrocytoma","type":"Histopathological subtype"},{"orpha":251598,"name":"Protoplasmic astrocytoma","type":"Histopathological subtype"}],"genes":[],"genes_via_subtypes":[{"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"],"via":{"orpha":251598,"name":"Protoplasmic astrocytoma"}},{"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"],"via":{"orpha":251601,"name":"Fibrillary astrocytoma"}},{"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"],"via":{"orpha":251604,"name":"Gemistocytic astrocytoma"}}],"inheritance":[],"onset":["Adolescent","Adult","Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.48,"area":"United States","status":"Validated","source":"Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID]"}],"phenotypes":null}