{"orpha":251962,"name":"Papillary glioneuronal tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PGNT","Pseudopapillary ganglioglioneurocytoma","Pseudopapillary neurocytoma with glial differentiation"],"definition":"A rare mixed neuronal-glial tumor characterized by a supratentorial space-occupying lesion in periventricular location, often with prominent cystic change. The histological hallmark of this low-grade neoplasm is its pseudopapillary appearance with a single layer of cuboidal cells around hyalinized blood vessels, associated with sheets or focal collections of neuronal cells. Clinical presentation is variable and non-specific, most frequently with headache and seizures. Prognosis is favorable after complete resection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-251962/","orphanet_url":"https://www.orpha.net/en/disease/detail/251962","names":{"fr":{"name":"Tumeur glioneuronale papillaire","synonyms":["Ganglioglioneurocytoma pseudopapillaire","Neurocytome pseudopapillaire à différenciation gliale","PGNT"],"definition":"Tumeur mixte neuronale-gliale rare, caractérisée par une lésion expansive supratentorielle en position périventriculaire, souvent avec un changement kystique proéminent. La caractéristique histologique de ce néoplasme de bas grade est l'aspect pseudo-papillaire avec une couche unique de cellules cuboïdales disposées autour de vaisseaux sanguins hyalinisés, associée à des couches ou à des concentrations de cellules neuronales. La présentation clinique est variable et non spécifique, avec le plus souvent des céphalées et des crises convulsives. Le pronostic est favorable après une résection complète."},"de":{"name":"Tumor, glioneuraler papillärer","synonyms":["Ganglioglioneurozytom, pseudopapilläres","Neurozytom, pseudopapilläres, mit glialer Differenzierung","PGNT"],"definition":"Ein seltener gemischter neuronal-glialer Tumor, der durch eine supratentorielle, raumfordernde Läsion in periventrikulärer Lage gekennzeichnet ist, oft mit auffälligen zystischen Veränderungen. Das histologische Merkmal dieser niedriggradigen Neoplasie ist ihr pseudopapilläres Erscheinungsbild mit einer einzelnen Schicht quaderförmiger Zellen um hyalinisierte Blutgefäße, die mit Blättern oder fokalen Ansammlungen neuronaler Zellen verbunden sind. Das klinische Bild ist variabel und unspezifisch, am häufigsten treten Kopfschmerzen und Krampfanfälle auf. Die Prognose ist nach vollständiger Resektion günstig."},"es":{"name":"Tumor glioneural papilar","synonyms":["Ganglioglioneurocitoma pseudopapilar","Neurocitoma pseudopapilar con diferenciación glial","PGNT"],"definition":"Es un tumor mixto neuronal-glial poco frecuente caracterizado por una lesión supratentorial ocupante de espacio de localización periventricular, que asocia, a menudo, cambios quísticos prominentes. La característica histologica distintiva de esta neoplasia de bajo grado es su apariencia pseudopapilar con una única capa de células cuboideas alrededor de los vasos sanguíneos hialinizados, asociada a colecciones laminares o focales de neuronas. La presentación clínica es variable e inespecífica, presentándose con mayor frecuencia con cefalea y crisis epilépticas. El pronóstico es favorable después de la resección completa."},"it":{"name":"Tumore glioneurale papillare","synonyms":["Ganglioglioneurocitoma pseudopapillare","Neurocitoma pseudopapillare con differenziazione gliale","PGNT"],"definition":"Si tratta di una neoplasia mista neuronale-gliale rara, caratterizzata da una lesione sopratentoriale voluminosa a localizzazione periventricolare, spesso con un'evidente alterazione cistica. Dal punto di vista istologico, questa neoplasia di basso grado è caratterizzata da un aspetto pseudopapillare con un singolo strato di cellule cuboidali attorno ai vasi sanguigni ialinizzati, associato a strati o raggruppamenti focali di cellule neuronali. Il quadro clinico è variabile e aspecifico, e spesso comprende la cefalea e le crisi epilettiche. Dopo la resezione completa la prognosi è favorevole."},"nl":{"name":"Papillaire glioneuronale tumor","synonyms":["PGNT","Pseudopapillair ganglioglioneurocytoom","Pseudopapillair neurocytoom met gliale differentiatie"],"definition":"Een zeldzame gemengde neuronale-gliale tumor, gekarakteriseerd door een supratentoriële, ruimte-innemende laesie met periventriculaire locatie, en vaak met prominente cystische verandering. Het typische histologische kenmerk van dit laaggradig neoplasma is het pseudopapillaire uiterlijk met een enkele laag van cuboïdale cellen rond gehyaliniseerde bloedvaten, geassocieerd met lagen of focale verzamelingen van zenuwcellen. De klinische presentatie is variabel en aspecifiek, meestal met hoofdpijn en insulten. De prognose is gunstig na volledige excisie."},"pt":{"name":"Tumor glioneuronal papilar","synonyms":["Ganglioglioneurocitoma pseudopapilar","Neurocitoma pseudopapilar com diferenciação glial","PGNT"]},"pl":{"name":"Guz brodawkowaty glioneuronalny","synonyms":["PGNT","Pseudopapillary ganglioglioneurocytoma","Pseudopapillary neurocytoma with glial differentiation"]},"cs":{"name":"Papilární glioneurální tumor","synonyms":["PGNT","Pseudopapilární ganglioglioneurocytom","Pseudopapilární neurocytom s gliální diferenciací"]},"uk":{"name":"Папілярна гліоневрональна пухлина","synonyms":["PGNT","Псевдопапілярна гангліогліоневроцитома","Псевдопапілярна нейроцитома з гліальним диференціюванням"],"definition":"A rare mixed neuronal-glial tumor characterized by a supratentorial space-occupying lesion in periventricular location, often with prominent cystic change. The histological hallmark of this low-grade neoplasm is its pseudopapillary appearance with a single layer of cuboidal cells around hyalinized blood vessels, associated with sheets or focal collections of neuronal cells. Clinical presentation is variable and non-specific, most frequently with headache and seizures. Prognosis is favorable after complete resection."},"zh":{"name":"乳头状神经胶质瘤","synonyms":["假乳头神经细胞瘤伴胶质分化"],"definition":"一种罕见的以脑室周围幕上占位性病变为特征的神经元-神经胶质混合瘤，常伴有明显的囊性改变。这种低度恶性肿瘤的组织学特征是假乳头状外观，透明血管周围有单层立方细胞，与神经细胞片状或局灶性聚集有关。临床表现多变且无特异性，最常见的是头痛和癫痫发作。完全切除后预后良好。"},"tr":{"name":"Papiller glionöronal tümör","synonyms":["PGNT","Psödopapiller ganglioglionörositoma","Glial farklılaşma ile seyreden psödopapiller nörositoma"]}},"xrefs":[{"source":"ICD-10","code":"D33.0","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"2A00.21","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"433042746"},{"source":"ICD-11","code":"XH3XU4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1594631904"},{"source":"MONDO","code":"0016735","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2985174","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":[],"prevalence":[],"phenotypes":null}