{"orpha":251937,"name":"Gangliocytoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare, mixed neuronal-glial tumor characterized by slow growth and irregular arrangement of neoplastic ganglion cells (large, multipolar dysplastic neurons) within stroma composed of non-neoplastic glial elements. Most commonly it occurs in temporal lobe, but it can be located throughout central nervous system. Clinical manifestations vary depending on the location and include seizures, increased intracranial pressure, cerebellar signs and focal neurologic deficits. Memory disturbances, cranial nerve palsies and psychiatric symptoms have also been reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251937/","orphanet_url":"https://www.orpha.net/en/disease/detail/251937","names":{"fr":{"name":"Gangliocytome","definition":"Tumeur mixte neuronale-gliale rare caractérisée par une croissance lente et une disposition irrégulière de cellules ganglionnaires néoplasiques (grands neurones dysplasiques multipolaires) dans un stroma composé d'éléments gliaux non néoplasiques. Se formant le plus souvent dans le lobe temporal, la tumeur peut être localisée dans l'ensemble du système nerveux central. Les manifestations cliniques varient en fonction de la localisation et comprennent des crises convulsives, une augmentation de la pression intracrânienne, des signes cérébelleux et des déficits neurologiques focaux. Des troubles de la mémoire, une paralysie des nerfs crâniens et des symptômes psychiatriques ont également été rapportés."},"de":{"name":"Gangliozytom"},"es":{"name":"Gangliocitoma","definition":"Es un tumor mixto glioneuronal poco frecuente, caracterizado por un crecimiento lento y una disposición irregular de las células ganglionares neoplásicas (neuronas multipolares grandes displásicas) dentro del estroma compuesto por elementos gliales no neoplásicos. Ocurre generalmente en el lóbulo temporal, pero puede estar localizado en todo el sistema nervioso central. Las manifestaciones clínicas varían según la ubicación e incluyen convulsiones, aumento de la presión intracraneal, signos cerebelosos y deficiencias neurológicas focales. También se han notificado alteraciones de la memoria, parálisis de nervios craneales y síntomas psiquiátricos."},"it":{"name":"Gangliocitoma","definition":"Il gangliocitoma è una neoplasia mista neuronale-gliale rara, caratterizzata da crescita lenta e un'organizzazione irregolare delle cellule ganglionari neoplastiche (neuroni displastici multipolari di grandi dimensioni) con stroma composto da elementi gliali non neoplastici. Di solito si localizza nel lobo temporale, ma può colpire tutto il sistema nervoso centrale. I segni clinici variano a seconda della localizzazione e comprendono le crisi epilettiche, l'aumento della pressione intracranica, i segni cerebellari e i deficit neurologici focali. Può associarsi a deficit mnemonici, paralisi dei nervi cranici e sintomi psichiatrici."},"nl":{"name":"Gangliocytoom","definition":"Gangliocytoom is een zeldzame, gemengde neuronale-gliale tumor die wordt gekarakteriseerd door trage groei en onregelmatige schikking van neoplastische ganglioncellen (grote, multipolaire dysplastische neuronen) in stroma bestaande uit niet-neoplastische gliale elementen. Deze tumor komt meestal voor in een temporale kwab, maar kan zich eender waar in het centrale zenuwstelsel situeren. Klinische manifestaties variëren afhankelijk van de locatie en omvatten insulten, verhoogde intracraniële druk, cerebellaire tekenen en focale neurologische defecten. Geheugenstoornissen, hersenzenuwverlammingen en psychiatrische symptomen werden ook reeds gerapporteerd."},"pt":{"name":"Gangliocitoma"},"pl":{"name":"Zwojak","definition":"*Zwojak jest rzadkim, mieszanym guzem neuronalno-glejowym, który charakteryzuje się powolnym wzrostem i nieregularnym układem neoplastycznych nowotworowych komórek zwojowych (dużych, wielobiegunowych neuronów dysplastycznych) w obrębie zrębu zbudowanego z nienowotworowych elementów glejowych. Najczęściej występuje w płacie skroniowym, ale może być zlokalizowany w całym ośrodkowym układzie nerwowym. Objawy kliniczne zależą od lokalizacji i obejmują drgawki, podwyższone ciśnienie śródczaszkowe, objawy móżdżkowe i ogniskowe deficyty neurologiczne. Opisywano również zaburzenia pamięci, porażenia nerwów czaszkowych i zaburzenia psychiczne."},"cs":{"name":"Gangliocytom"},"uk":{"name":"Гангліоцитома","definition":"A rare, mixed neuronal-glial tumor characterized by slow growth and irregular arrangement of neoplastic ganglion cells (large, multipolar dysplastic neurons) within stroma composed of non-neoplastic glial elements. Most commonly it occurs in temporal lobe, but it can be located throughout central nervous system. Clinical manifestations vary depending on the location and include seizures, increased intracranial pressure, cerebellar signs and focal neurologic deficits. Memory disturbances, cranial nerve palsies and psychiatric symptoms have also been reported."},"zh":{"name":"神经节细胞瘤","definition":"神经节细胞瘤是一种罕见的神经元-胶质混合性肿瘤，其特征是生长缓慢，在由非肿瘤性胶质成分构成的间质中排列不规则的肿瘤性神经节细胞(发育异常的较大的多极神经元)。最常见的是发生在颞叶，但也可以分布在整个中枢神经系统。临床表现因部位不同而不同，包括癫痫发作、颅内压升高、小脑体征和局灶性神经功能障碍。记忆障碍、脑神经麻痹和精神症状也有报道。"},"tr":{"name":"Gangliositom"}},"xrefs":[{"source":"ICD-10","code":"D36.1","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2A00.21","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1996187773"},{"source":"ICD-11","code":"XH6KA6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"575417126"},{"source":"MONDO","code":"0016730","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5779630","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10638","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":251934,"name":"Mixed neuronal-glial tumor","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adolescent","Childhood","Infancy"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"18798534[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-29","source":"25259876[PMID]_2369716[PMID]_24430434[PMID]_6423659[PMID]_3022544[PMID]_12060349[PMID]_2512034[PMID]_10470826[PMID]_6707754[PMID]_2927689[PMID]_19789938[PMID]_18651251[PMID]_12622135[PMID]_9055303[PMID]_8264895[PMID]_6631503[PMID]_6955631[PMID]_7252559[PMID]_26314658[PMID]_7584515[PMID]_6955631[PMID]_18297623[PMID]_17460525[PMID]_15690130[PMID]_15739067[PMID]_11373931[PMID]_1767634[PMID]_8246055[PMID]","terms":[{"hpo":"HP:0003005","label":"Ganglioneuroma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012377","label":"Hemianopia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100006","label":"Neoplasm of the central nervous system","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010302","label":"Spinal cord tumor","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002363","label":"Abnormal brainstem morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040086","label":"Abnormal prolactin level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012503","label":"Abnormality of the pituitary gland","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005616","label":"Accelerated skeletal maturation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011749","label":"Adrenocorticotropic hormone excess","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000141","label":"Amenorrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030018","label":"Decreased female libido","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002460","label":"Distal muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000845","label":"Elevated circulating growth hormone concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007359","label":"Focal-onset seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000975","label":"Hyperhidrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000802","label":"Impotence","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011761","label":"Pituitary null cell adenoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002650","label":"Scoliosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001317","label":"Abnormal cerebellum morphology","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000726","label":"Dementia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001262","label":"Excessive daytime somnolence","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006767","label":"Pituitary prolactin cell adenoma","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002591","label":"Polyphagia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0003396","label":"Syringomyelia","frequency":"Very rare (<4-1%)","criteria":null}]}}