{"orpha":251927,"name":"Extraventricular neurocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["EVN"],"definition":"Extraventricular neurocytoma (EVN), a variant of central neurocytoma, is a rare neuronal neoplasm, composed of round cells with neuronal differentiation, which is located outside of the ventricular system, usually within the spinal cord or cerebral hemispheres and that manifests with headache, nausea, vomiting, complex partial seizures or focal neurological deficits. In some cases it may exhibit atypical features consistent with aggressive clinical behavior.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251927/","orphanet_url":"https://www.orpha.net/en/disease/detail/251927","names":{"fr":{"name":"Neurocytome extraventriculaire","synonyms":["EVN"],"definition":"Le neurocytome extraventriculaire, un variant du neurocytome central est un néoplasme neuronal rare, composé de cellules arrondies avec une différenciation neuronale. Il est situé en dehors du système ventriculaire, généralement au sein de la moelle épinière ou des hémisphères cérébraux, et se manifeste par des céphalées, des nausées, des vomissements, des convulsions partielles complexes ou des déficits neurologiques focaux. Dans certains cas, il peut présenter des caractéristiques atypiques consistantes avec un comportement clinique agressif."},"de":{"name":"Neurozytom, extraventrikuläres","synonyms":["EVN"]},"es":{"name":"Neurocitoma extraventricular","synonyms":["EVN"],"definition":"El neurocitoma extraventricular (EVN), una variante de neurocitoma central (consulte este término), es una neoplasia neuronal poco frecuente, compuesta de células redondas con diferenciación neuronal, que está localizado fuera del sistema ventricular, normalmente en la médula espinal o los hemisferios cerebrales. Se manifiesta con dolor de cabeza, náuseas, vómitos, convulsiones parciales complejas o déficits neurológicos focales. En algunos casos puede mostrar rasgos atípicos consistentes con un comportamiento clínico agresivo."},"it":{"name":"Neurocitoma extraventricolare","definition":"Il neurocitoma extraventricolare (EVN), una variante del neurocitoma centrale (si veda questo termine), è una neoplasia neuronale rara, contenente cellule rotonde con differenziazione neuronale. Si localizza in un sito diverso dal sistema ventricolare, di solito all'interno del midollo spinale o degli emisferi cerebrali. Si manifesta con cefalea, nausea, vomito, crisi epilettiche parziali complesse o deficit neurologici focali. In alcuni casi, possono essere presenti segni atipici, coerenti con un comportamento clinico aggressivo."},"nl":{"name":"Extraventriculair neurocytoom","synonyms":["EVN"],"definition":"Extraventriculair neurocytoom (EVN), een variant van centraal neurocytoom (zie deze term), is een zeldzaam neuronaal neoplasma dat bestaat uit ronde cellen met neuronale differentiatie, gesitueerd is buiten het ventrikelsysteem (meestal in het ruggenmerg of cerebrale hemisferen), en zich manifesteert met hoofdpijn, misselijkheid, braken, complexe partiële insulten of focale neurologische defecten. In sommige gevallen kunnen atypische kenmerken naar voren komen die consistent zijn met agressief klinisch gedrag."},"pt":{"name":"Neurocitoma extraventricular"},"pl":{"name":"Nerwiak komórkowy pozakomorowy","synonyms":["EVN"]},"cs":{"name":"Extraventrikulární neurocytom","synonyms":["EVN"]},"uk":{"name":"Позашлуночкова нейроцитома","synonyms":["EVN"],"definition":"Extraventricular neurocytoma (EVN), a variant of central neurocytoma (see this term), is a rare neuronal neoplasm, composed of round cells with neuronal differentiation, which is located outside of the ventricular system, usually within the spinal cord or cerebral hemispheres and that manifests with headache, nausea, vomiting, complex partial seizures or focal neurological deficits. In some cases it may exhibit atypical features consistent with aggressive clinical behavior."},"zh":{"name":"脑室外神经细胞瘤","synonyms":["EVN"],"definition":"室外神经细胞瘤(EVN)是中枢神经细胞瘤（见该词条）的变种，是一种罕见的神经细胞肿瘤，由具有神经元分化的圆形细胞组成，位于脑室系统外，通常位于脊髓或大脑半球内，表现为头痛、恶心、呕吐、复杂的部分性癫痫或局灶性神经功能障碍。在某些情况下，它可能表现出与攻击性临床行为一致的非典型特征。"},"tr":{"name":"Ekstraventriküler nörositom","synonyms":["EVN"]}},"xrefs":[{"source":"ICD-10","code":"C72.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A00.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"XH2HS1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"445204761"},{"source":"MONDO","code":"0016727","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2985175","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251924,"name":"Neuronal tumor","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}