{"orpha":251931,"name":"Cerebellar liponeurocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Cerebellar liponeurocytoma (cLPN) is a rare slow growing neuronal tumor seen more frequently in females than males, occurring most commonly in the cerebellum but occasionally in the supratentorial compartment or the fourth ventricle and presenting in the 4th to 6th decade of life with symptoms of dizziness, headache and gait instability. It often has a high rate of local recurrence.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251931/","orphanet_url":"https://www.orpha.net/en/disease/detail/251931","names":{"fr":{"name":"Liponeurocytome cérébelleux","definition":"Le liponeurocytome cérébelleux est une tumeur neuronale rare, lentement progressive observée plus souvent chez les femmes que chez les hommes. Il survient le plus souvent dans le cervelet, mais occasionnellement, dans le compartiment supratentoriel ou le quatrième ventricule, et se présente entre la quatrième et la sixième décennie par des vertiges, des céphalées et une démarche instable. Le taux de récurrence locale est souvent élevé."},"de":{"name":"Liponeurozytom, zerebelläres"},"es":{"name":"Liponeurocitoma cerebeloso","definition":"El liponeurocitoma cerebeloso (cLPN) es un tumor neuronal poco frecuente de crecimiento lento, visto con mayor frecuencia en mujeres que en varones. Generalmente afecta al cerebelo pero en ocasiones se presenta en el compartimento supratentorial o en el cuarto ventrículo. Aparece en la 4ª a 6ª década de vida con síntomas que incluyen mareos, dolor de cabeza, e inestabilidad en la marcha. A menudo presenta una elevada tasa de recurrencia local."},"it":{"name":"Liponeurocitoma cerebellare","definition":"Il liponeurocitoma cerebellare (cLPN) è un tumore raro neuronale a progressione lenta, più comune nelle donne che nei maschi. Si manifesta di solito nel cervelletto e, raramente, nel comparto sovratentoriale del quarto ventricolo. Esordisce tra la quarta e la sesta decade di vita, con intorpidimento, cefalea e andatura instabile. Spesso ha un elevato tasso di ricorrenza locale."},"nl":{"name":"Cerebellair liponeurocytoom","definition":"Cerebellair liponeurocytoom (cLPN) is een zeldzame traag groeiende neuronale tumor die meer wordt waargenomen bij vrouwen dan bij mannen, doorgaans voorkomt in de kleine hersenen maar soms ook in het supratentoriale compartiment van het vierde ventrikel, en tot uiting komt in het 4de tot het 6de levensdecennium met duizeligheid, hoofdpijn en instabiele tred als symptomen. Vaak is er een hoge graad van lokale terugval."},"pt":{"name":"Liponeurocitoma cerebeloso"},"pl":{"name":"Tłuszczakonerwiak komórkowy móżdżku"},"cs":{"name":"Liponeurocytom mozečku"},"uk":{"name":"Ліпонейроцитома мозочка","definition":"Cerebellar liponeurocytoma (cLPN) is a rare slow growing neuronal tumor seen more frequently in females than males, occurring most commonly in the cerebellum but occasionally in the supratentorial compartment or the fourth ventricle and presenting in the 4th to 6th decade of life with symptoms of dizziness, headache and gait instability. It often has a high rate of local recurrence."},"zh":{"name":"小脑磷脂神经细胞瘤","definition":"小脑脂肪神经细胞瘤(CLPN)是一种罕见的生长缓慢的神经元肿瘤，女性多于男性，最常发生在小脑，但偶尔发生在幕上隔室或第四脑室，在40-60岁时发病，表现出头晕、头痛和步态不稳的症状。它的局部复发率往往很高。"},"tr":{"name":"Serebellar liponörositom"}},"xrefs":[{"source":"ICD-10","code":"D33.1","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"2A00.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"XH2GB0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"950838670"},{"source":"MONDO","code":"0006131","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1370507","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10642","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":["Not applicable"],"onset":["Adult"],"prevalence":[],"phenotypes":null}