{"orpha":251883,"name":"Medulloepithelioma of the central nervous system","type":"Disease","group":"Disorder","status":"inactive","flags":["Deprecated entity"],"synonyms":[],"definition":null,"note":"This entity has been excluded from the Orphanet nomenclature of rare diseases and moved to Embryonal tumor with multilayered rosettes","url":"https://raremap.alftech.space/d/ORPHA-251883/","orphanet_url":"https://www.orpha.net/en/disease/detail/251883","names":{"fr":{"name":"Médulloépithéliome du système nerveux central"},"de":{"name":"Medulloepitheliom des Zentralnervensystems"},"es":{"name":"Meduloepitelioma del sistema nervioso central"},"it":{"name":"Medulloepitelioma del sistema nervoso centrale"},"nl":{"name":"Medullo-epithelioom van centraal zenuwstelsel"},"pt":{"name":"Medulo-epitelioma"},"pl":{"name":"Nabłoniak rdzeniakowy"},"cs":{"name":"Meduloepiteliom"},"uk":{"name":"Медулоепітеліома центральної нервової системи"},"zh":{"name":"中枢神经系统髓质上皮瘤","definition":"中枢神经系统髓上皮瘤是一种罕见的原始神经外胚层肿瘤，其特征是肿瘤神经上皮呈乳头状、管状和小梁状排列，类似于胚胎神经管，最常见于大脑半球的脑室周围区域，但在脑干和小脑也有报道。它通常在童年时表现为头痛、恶心、呕吐、面神经麻痹和/或小脑性共济失调，通常具有进行性病程、高度恶性表现和不良预后。还观察到听力和视力的丧失。"},"tr":{"name":"Merkezi sinir sisteminin medulloepitelyoması"}},"xrefs":[{"source":"UMLS","code":"C5231013","qualifier":"E","icd_relation":null,"validated":true}],"associations":[{"type":"Moved to","orpha":656417,"name":"Embryonal tumor with multilayered rosettes"}],"incoming_associations":[],"preferential_parent":null,"parents":[],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}