{"orpha":251589,"name":"Astrocytoma grade 3","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Anaplastic astrocytoma"],"definition":"A rare, high-grade, malignant glial tumor, histologically characterized by abundance of pleomorphic astrocytes and multiple mitotic figures, often associated with diffuse infiltration of the surrounding tissue, considerable edema and mass effect and involvement of the contralateral brain. Depending on the primary localization of the tumor, patients can present with signs of raised intracranial pressure (headache, vomiting, papilledema), seizures, progressive neurological deficits, and/or behavioral changes. The tumor is most commonly localized in the frontal and temporal lobes, brain stem and spinal cord.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251589/","orphanet_url":"https://www.orpha.net/en/disease/detail/251589","names":{"fr":{"name":"Astrocytome de grade 3","synonyms":["Astrocytome anaplasique"],"definition":"L'astrocytome anaplasique est une tumeur gliale maligne rare, de grade élevé, caractérisée sur le plan histologique par d'abondants astrocytes pléomorphes et de nombreuses figures mitotiques, souvent associés à une infiltration diffuse du tissu environnant, un oedème et un effet de masse très importants, et l'atteinte du cerveau contralatéral. En fonction de la localisation primaire de la tumeur, les patients peuvent présenter des signes d'augmentation de la pression intracrânienne (céphalées, vomissements, papilloedème), des convulsions, des troubles neurologiques progressifs et/ou des changements de comportement. La tumeur est le plus souvent située dans les lobes frontaux et temporaux, le tronc cérébral et la moelle épinière."},"de":{"name":"Astrozytom Grad 3","synonyms":["Astrozytom, anaplastisches"],"definition":"Ein seltener, hochgradiger, maligner Glia-Tumor, histologisch gekennzeichnet durch eine Fülle pleomorpher Astrozyten und multiplen mitotischen Zellen, oft verbunden mit diffuser Infiltration des umgebenden Gewebes, erheblichem Ödem und Masseneffekt sowie Beteiligung des kontralateralen Gehirns. Abhängig von der primären Lokalisation des Tumors können sich die Patienten mit Zeichen eines erhöhten Hirndrucks (Kopfschmerzen, Erbrechen, Papillenödem), Krampfanfällen, progressiven neurologischen Defiziten und/oder Verhaltensänderungen präsentieren. Der Tumor ist am häufigsten in den Frontal- und Temporallappen, im Hirnstamm und im Rückenmark lokalisiert."},"es":{"name":"Astrocitoma de grado 3","synonyms":["Astrocitoma anaplásico"],"definition":"Es un tumor glial maligno de alto grado y poco frecuente caracterizado histológicamente por la abundancia de astrocitos pleomórficos y múltiples figuras mitóticas y, a menudo, asociado con infiltración difusa del tejido circundante, edema y efecto masa considerables con afectación del cerebro contralateral. Dependiendo de la localización primaria del tumor, los afectados pueden presentar signos de aumento de la presión intracraneal (cefalea, vómitos, papiledema), convulsiones, deficiencia neurológica progresiva, y/o cambios de la conducta. Por lo general, el tumor se localiza en los lóbulos frontales y temporales, tronco cerebral y médula espinal."},"it":{"name":"Astrocitoma di grado 3","synonyms":["Astrocitoma anaplastico"],"definition":"L'astrocitoma anaplastico è un tumore gliale raro ad elevato grado di malignità; dal punto di vista istologico è caratterizzato da un'abbondanza di astrociti pleomorfi e numerose figure mitotiche, spesso associate ad un'infiltrazione diffusa nei tessuti circostanti, edema ed effetto massa significativo e interessamento dell'area controlaterale del cervello. A seconda della localizzazione primaria del tumore, i pazienti possono presentare i segni di un aumento della pressione intracranica (cefalea, vomito, papilledema), crisi epilettiche, deficit neurologici progressivi e/o cambiamenti del comportamento. Per lo più il tumore si localizza nel lobo frontale e temporale, nel tronco encefalico e nel midollo spinale."},"nl":{"name":"Astrocytoom graad 3","synonyms":["Anaplastisch astrocytoom"],"definition":"Een zeldzame, hooggradige, kwaadaardige gliale tumor, histologisch gekarakteriseerd door een overvloed aan pleomorfe astrocyten en meervoudige mitotische spoelfiguren, vaak geassocieerd met diffuse infiltratie van omliggend weefsel, aanzienlijk oedeem en massa-effect, en betrokkenheid van de contralaterale hersenen. Afhankelijk van de primaire lokalisatie van de tumor kunnen patiënten symptomen van verhoogde intracraniële druk (hoofdpijn, braken, papiloedeem), insulten, progressieve neurologische defecten, en/of gedragsveranderingen vertonen. De tumor is meestal gesitueerd in de frontale en temporale kwabben, de hersenstam en het ruggenmerg."},"pt":{"name":"Astrocitoma anaplásico"},"pl":{"name":"Gwiaździak anaplastyczny","synonyms":["Anaplastic astrocytoma"]},"cs":{"name":"Anaplastický astrocytom","synonyms":["Anaplastic astrocytoma"]},"uk":{"name":"Анапластична астроцитома","definition":"A rare, high-grade, malignant glial tumor, histologically characterized by abundance of pleomorphic astrocytes and multiple mitotic figures, often associated with diffuse infiltration of the surrounding tissue, considerable edema and mass effect and involvement of the contralateral brain. Depending on the primary localization of the tumor, patients can present with signs of raised intracranial pressure (headache, vomiting, papilledema), seizures, progressive neurological deficits, and/or behavioral changes. The tumor is most commonly localized in the frontal and temporal lobes, brain stem and spinal cord."},"zh":{"name":"间变性星形细胞瘤","definition":"一种罕见的高级别恶性胶质瘤，组织学上以丰富的多形性星形细胞和多种有丝分裂像为特征，常伴有周围组织的弥漫性浸润，相当大的水肿和肿块效应，并累及对侧大脑。根据肿瘤的主要部位，患者可能出现颅内压升高(头痛、呕吐、乳头水肿)、癫痫发作、进行性神经功能缺损和/或行为改变的迹象。肿瘤最常见的部位是额叶和颞叶、脑干和脊髓。"},"tr":{"name":"Anaplastik astrositom"}},"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":"595864231"},{"source":"ICD-11","code":"XH96C7","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1593883407"},{"source":"MONDO","code":"0016684","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334579","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"5860","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10002224","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251561,"name":"High-grade astrocytoma","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":"24993250[PMID]_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":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"United States","status":"Validated","source":"Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID]"}],"phenotypes":null}