{"orpha":252046,"name":"Meningeal melanocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare nervous system tumor characterized by a benign pigmented space-occupying lesion derived from leptomeningeal melanocytes. Symptoms typically show insidious onset and are related to the mass effect on adjacent tissues. Depending on the location of the tumor, they include focal neurological deficits, increased intracranial pressure, seizures, and spinal cord compression, among others. Although the tumor may behave aggressively, prognosis is good after complete surgical resection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-252046/","orphanet_url":"https://www.orpha.net/en/disease/detail/252046","names":{"fr":{"name":"Mélanocytome méningé","definition":"Tumeur rare du système nerveux caractérisée par une lésion pigmentée bénigne dérivée de mélanocytes des leptoméninges se développant aux dépends des tissus. Les symptômes apparaissent généralement de manière insidieuse et sont liés à l'effet de la masse tumorale sur les tissus adjacents. En fonction de la localisation de la tumeur, les signes cliniques sont, entre autres, des déficits neurologiques focaux, une augmentation de la pression intracrânienne, des crises convulsives et une compression de la moelle épinière. Bien que la tumeur puisse être agressive, le pronostic est bon après une résection chirurgicale complète."},"de":{"name":"Melanozytom, meningeales","definition":"Ein seltener Tumor des Nervensystems, der durch eine gutartige pigmentierte, raumfordernde Läsion gekennzeichnet ist, die von leptomeningealen Melanozyten abstammt. Die Symptome treten typischerweise schleichend auf und hängen mit der Gewebeverdichtung in den angrenzenden Geweben zusammen. Je nach Lage des Tumors können sie u. a. fokale neurologische Defizite, erhöhten Hirndruck, Krampfanfälle und Rückenmarkskompression umfassen. Obwohl sich der Tumor aggressiv verhalten kann, ist die Prognose nach vollständiger chirurgischer Resektion gut."},"es":{"name":"Melanocitoma meníngeo","definition":"Es un tumor poco frecuente del sistema nervioso caracterizado por una lesión ocupante de espacio pigmentada benigna derivada de melanocitos leptomeníngeos. Por lo general, los síntomas muestran un inicio insidioso, y están asociados al efecto masa en los tejidos adyacentes. Dependiendo de la localización tumoral, incluyen déficits neurológicos focales, aumento de la presión intracraneal, crisis epilépticas y compresión de la médula espinal, entre otros. Aunque el tumor puede presentar un comportamiento agresivo, el pronóstico es favorable después de la resección quirúrgica completa."},"it":{"name":"Melanocitoma meningeo","definition":"Si tratta di un tumore raro del sistema nervoso, caratterizzato da una lesione benigna pigmentata espansiva che origina dai melanociti leptomeningei. Di solito l'esordio è insidioso; i sintomi dipendono dagli effetti della massa sui tessuti circostanti e, a seconda della localizzazione del tumore, comprendono i deficit neurologici focali, l'aumento della pressione intracranica, le crisi epilettiche e la compressione del midollo spinale. Sebbene il tumore possa essere aggressivo, dopo la resezione chirurgica completa la prognosi è buona."},"nl":{"name":"Meningeaal melanocytoom","definition":"Een zeldzame tumor van zenuwstelsel, gekarakteriseerd door een goedaardige, gepigmenteerde, ruimte-innemende laesie afgeleid van leptomeningeale melanocyten. Symptomen vertonen doorgaans een sluipend begin en zijn gerelateerd aan het massa-effect op aangrenzende weefsels. Afhankelijk van de locatie van de tumor gaat het onder meer om focale neurologische functiestoornissen, verhoogde intracraniële druk, insulten, en compressie van ruggenmerg. hOEWEL De tumor ZICH agressief kan gedragen, is de prognose goed na volledige chirurgische excisie."},"pt":{"name":"Melanocitoma meníngeo"},"pl":{"name":"Melanocytoma opon mózgowych"},"cs":{"name":"Meningeální melanocytom"},"uk":{"name":"Менінгеальна меланоцитома","definition":"A rare nervous system tumor characterized by a benign pigmented space-occupying lesion derived from leptomeningeal melanocytes. Symptoms typically show insidious onset and are related to the mass effect on adjacent tissues. Depending on the location of the tumor, they include focal neurological deficits, increased intracranial pressure, seizures, and spinal cord compression, among others. Although the tumor may behave aggressively, prognosis is good after complete surgical resection."},"zh":{"name":"脑膜黑素细胞瘤","definition":"一种罕见的神经系统肿瘤，其特征是来源于软脑膜黑素细胞的良性色素占位性病变。症状通常表现为潜伏性发作，并与邻近组织的肿块效应有关。根据肿瘤的位置，它们包括局灶性神经功能障碍、颅内压升高、癫痫发作和脊髓压迫等。虽然肿瘤可能表现为侵袭性，但完全手术切除后预后良好。"},"tr":{"name":"Meninjiyal melanositom"}},"xrefs":[{"source":"ICD-10","code":"D32.9","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A01.0Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"XH2RY7","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"473991947"},{"source":"MONDO","code":"0016746","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1266113","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":252028,"name":"Primary melanocytic tumor of central nervous system","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}