{"orpha":73256,"name":"Central neurocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Central neurocytoma is a very rare brain tumor of young adults (over 100 cases reported worldwide). It is typically found in the lateral ventricles and occasionally in the third ventricle. Symptoms are those of increased intracranial pressure: headache, nausea and vomiting, drowsiness, vision problems and mental changes. Total removal of the tumor is the therapy of choice. Post-operative prognosis is generally good.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-73256/","orphanet_url":"https://www.orpha.net/en/disease/detail/73256","names":{"fr":{"name":"Neurocytome central","definition":"Le neurocytome central est une tumeur cérébrale très rare de l'adulte jeune (une centaine de cas rapportée dans le monde). Elle est typiquement localisée dans les ventricules latéraux et parfois dans le troisième ventricule. Les symptômes correspondent à ceux d'une hypertension endocrânienne : céphalées, nausées et vomissements, somnolence, troubles de la vision et troubles de la conscience. L'exérèse complète de la tumeur est le traitement de choix. Le pronostic post-opératoire est généralement bon."},"de":{"name":"Neurozytom, zentrales","definition":"Das Zentrale Neurozytom ist ein sehr seltener Hirntumor junger Erwachsener."},"es":{"name":"Neurocitoma central","definition":"El neurocitoma central es un tumor cerebral muy poco frecuente que se presenta en el adulto joven (se han descrito unos 100 casos en todo el mundo). Se suele presentar en los ventrículos laterales y, ocasionalmente, en el tercer ventrículo. Los síntomas son los típicos del aumento de la presión intracraneal: cefalea, nauseas, vómitos, mareos, problemas de visión y cambios mentales. La terapia adecuada es la extracción completa del tumor. El pronóstico postoperatorio es generalmente bueno."},"it":{"name":"Neurocitoma centrale"},"nl":{"name":"Centraal neurocytoom","definition":"Centraal neurocytoom is een zeer zeldzame hersentumor bij jongvolwassenen (wereldwijd meer dan 100 gevallen gerapporteerd). Het wordt doorgaans aangetroffen in laterale ventrikels en soms in derde ventrikel. Symptomen zijn die van verhoogde intracraniële druk: hoofdpijn, misselijkheid en braken, slaperigheid, visuele problemen, en mentale veranderingen. Volledige verwijdering van de tumor draagt de voorkeur als therapie. De postoperatieve prognose is doorgaans goed."},"pt":{"name":"Neurocitoma central"},"pl":{"name":"Neurocytoma ośrodkowa"},"cs":{"name":"Centrální neurocytom"},"uk":{"name":"Центральна нейроцитома","definition":"Central neurocytoma is a very rare brain tumor of young adults (over 100 cases reported worldwide). It is typically found in the lateral ventricles and occasionally in the third ventricle. Symptoms are those of increased intracranial pressure: headache, nausea and vomiting, drowsiness, vision problems and mental changes. Total removal of the tumor is the therapy of choice. Post-operative prognosis is generally good."},"zh":{"name":"中枢神经细胞瘤"},"tr":{"name":"Santral nörositoma"}},"xrefs":[{"source":"ICD-10","code":"D33.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A00.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1247650801"},{"source":"MONDO","code":"0019134","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206719","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10641","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":251924,"name":"Neuronal tumor","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":500.0,"area":"Worldwide","status":"Validated","source":"23810386[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-02","source":"3776470[PMID]_15015671[PMID]","terms":[{"hpo":"HP:0000238","label":"Hydrocephalus","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0025354","label":"Abnormal cellular phenotype","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030047","label":"Abnormality of lateral ventricle","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010576","label":"Intracranial cystic lesion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000504","label":"Abnormality of vision","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001251","label":"Ataxia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002514","label":"Cerebral calcification","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008000","label":"Decreased corneal reflex","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002516","label":"Increased intracranial pressure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003487","label":"Babinski sign","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001254","label":"Lethargy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007021","label":"Pain insensitivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002172","label":"Postural instability","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000360","label":"Tinnitus","frequency":"Occasional (29-5%)","criteria":null}]}}