{"orpha":46135,"name":"Primary central nervous system lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PCNSL","Primary CNS lymphoma","Primary brain lymphoma"],"definition":"Primary central nervous system lymphoma (PCNSL) is a rare nervous system tumor, predominantly due to diffuse large B-cell lymphoma, that involves brain, leptomeninges, eyes, or rarely spinal cord, in the absence of systemic diffusion at the time of diagnosis. It is characterized by a solitary tumor that, depending on its location, can lead to a variety of symptoms such as headache, nausea, vomiting (and other signs of raised intracranial pressure), focal neurologic deficits, neuropsychiatric and ocular symptoms, seizures and personality changes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-46135/","orphanet_url":"https://www.orpha.net/en/disease/detail/46135","names":{"fr":{"name":"Lymphome primitif du système nerveux central","synonyms":["Lymphome cérébral primitif","Lymphome primitif du SNC","PCNSL"],"definition":"Tumeur rare du système nerveux, principalement due à un lymphome diffus à grandes cellules B, affectant le cerveau, les leptoméninges, les yeux ou, rarement, la moelle épinière, en l'absence de dissémination systémique au moment du diagnostic. Elle se caractérise par une tumeur solitaire laquelle, selon la localisation, peut entraîner divers symptômes tels que des maux de tête, des nausées, des vomissements (et d'autres signes d'augmentation de la pression intracrânienne), des déficits neurologiques focaux, des symptômes neuropsychiatriques et oculaires, des crises d'épilepsie et des changements de personnalité."},"de":{"name":"Primäres Lymphom des Zentralnervensystems","synonyms":["PZNSL","ZNS-Lymphom, primäres"],"definition":"Das primäre Lymphom des Zentralnervensystems (PCNSL) ist ein seltener Tumor des Nervensystems, der überwiegend auf ein diffuses großzelliges B-Zell-Lymphom zurückzuführen ist und das Gehirn, die Leptomeningen, die Augen oder selten das Rückenmark betrifft, wenn zum Zeitpunkt der Diagnose keine systemische Ausbreitung vorliegt. Es ist durch einen solitären Tumor gekennzeichnet, der je nach Lokalisation zu einer Vielzahl von Symptomen wie Kopfschmerzen, Übelkeit, Erbrechen (und anderen Zeichen eines erhöhten Hirndrucks), fokalen neurologischen Defiziten, neuropsychiatrischen und okulären Symptomen, Krampfanfällen und Persönlichkeitsveränderungen führen kann."},"es":{"name":"Linfoma primario del sistema nervioso central","synonyms":["Linfoma cerebral primario","Linfoma del SNC primario","PCNSL"],"definition":"Es un tumor poco frecuente del sistema nervioso debido principalmente a un linfoma difuso B de células grandes que afecta al cerebro, leptomeninges, ojos y, excepcionalmente, a la médula espinal, en ausencia de diseminación sistémica en el momento del diagnóstico. Se caracteriza por un tumor aislado que, según su localización, puede provocar diversos síntomas, tales como cefalea, náuseas, vómitos (y otros signos de aumento de la presión intracraneal), déficits neurológicos focales, síntomas neuropsiquiátricos y oculares, convulsiones y cambios de la personalidad."},"it":{"name":"Linfoma primitivo del sistema nervoso centrale","synonyms":["Linfoma cerebrale primitivo","Linfoma primitivo del CNS","PCNSL"],"definition":"Il linfoma primitivo del sistema nervoso centrale (PCNSL) è una neoplasia rara, per lo più secondaria ad un linfoma diffuso a grandi cellule B, che interessa il cervello, le leptomeningi, gli occhi, oppure, raramente, il midollo spinale, in assenza di una diffusione sistemica al momento della diagnosi. Esordisce con una massa solitaria che, a seconda della localizzazione, causa vari sintomi, come la cefalea, la nausea, il vomito e altri segni clinici secondari all'aumento della pressione intracranica, deficit neurologici focali, sintomi neuropsichiatrici e oculari, crisi epilettiche e cambiamenti della personalità."},"nl":{"name":"Primair lymfoom van centraal zenuwstelsel","synonyms":["PCZSL","Primair CZS-lymfoom","Primair hersenlymfoom"],"definition":"Primair lymfoom van het centrale zenuwstelsel (PCZSL) is een zeldzame tumor van het zenuwstelsel, voornamelijk als gevolg van diffuus grootcellig B-cellymfoom, met betrokkenheid van de hersenen, leptomeningen, ogen, of in zeldzame gevallen het ruggenmerg, in afwezigheid van systemische diffusie op het moment van de diagnose. Het wordt gekarakteriseerd door een solitaire tumor die, afhankelijk van de locatie, kan leiden tot verschillende symptomen, zoals hoofdpijn, misselijkheid, braken (en andere tekenen van een verhoogde intracraniële druk), focale neurologische defecten, neuropsychiatrische en oculaire symptomen, insulten en persoonlijkheidsveranderingen."},"pt":{"name":"Linfoma primário do sistema nervoso central","synonyms":["Linfoma primário CNS","Linfoma primário cerebral","PCNSL"]},"pl":{"name":"Pierwotny chłoniak ośrodkowego układu nerwowego","synonyms":["PCNSL","Pierwotny chłoniak CUN","Pierwotny chłoniak mózgu"],"definition":"*Pierwotny chłoniak ośrodkowego układu nerwowego jest rzadkim nowotworem układu nerwowego; głównie jest to chłoniak rozlany z dużych komórek B, który obejmuje mózg, opony mózgowe, oczy oraz rzadko rdzeń kręgowy, przy braku ogólnoustrojowego rozsiewu w momencie rozpoznania. Ma postać pojedynczego guza, który w zależności od lokalizacji może dawać różne objawy, np. bóle głowy, nudności, wymioty (i inne objawy podwyższonego ciśnienia wewnątrzczaszkowego), ogniskowe deficyty neurologiczne, objawy neuropsychiatryczne i oczne, drgawki i zmiany osobowości.inf brsup inf brsup"},"cs":{"name":"Primární lymfom centrálního nervového systému","synonyms":["PCNSL","Primární lymfom CNS","Primární mozkový lymfom"]},"uk":{"name":"Первинна лімфома центральної нервової системи","synonyms":["PCNSL","Первинна лімфома головного мозку","Первинна лімфома ЦНС"],"definition":"Primary central nervous system lymphoma (PCNSL) is a rare nervous system tumor, predominantly due to diffuse large B-cell lymphoma, that involves brain, leptomeninges, eyes, or rarely spinal cord, in the absence of systemic diffusion at the time of diagnosis. It is characterized by a solitary tumor that, depending on its location, can lead to a variety of symptoms such as headache, nausea, vomiting (and other signs of raised intracranial pressure), focal neurologic deficits, neuropsychiatric and ocular symptoms, seizures and personality changes."},"zh":{"name":"原发性中枢神经系统淋巴瘤","synonyms":["原发性脑淋巴瘤"]},"tr":{"name":"Primer santral sinir sistemi lenfoması","synonyms":["PCNSL","Primer CNS lenfoma","Primer beyin lenfoması"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A8Z","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1133193893"},{"source":"MONDO","code":"0002571","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0280803","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9318","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10036685","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":289644,"name":"Epstein-Barr virus-associated malignant lymphoproliferative disorder","classifications":[202]},{"orpha":279911,"name":"Primary organ-specific lymphoma","classifications":[194,202,233]},{"orpha":98062,"name":"Rare nervous system tumor","classifications":[181,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.44,"area":"United States","status":"Validated","source":"Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.26,"area":"Sweden","status":"Validated","source":"28983970[PMID]"}],"phenotypes":null}