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Linfoma primário do sistema nervoso central
ORPHA:46135CID-10 · C83.3CID-11 · 2A8ZDOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
Início adultaHerança Unknown
Também conhecida comoPCNSLLinfoma primário CNSLinfoma primário cerebralPCNSLCNS
Sinônimos clínicos: PCNSL · Linfoma primário CNS · Linfoma primário cerebral

Um linfoma não-Hodgkin ou de Hodgkin que surge no cérebro ou na medula espinhal como uma lesão primária. Não há evidência de linfoma fora do sistema nervoso central no momento do diagnóstico.

Em construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

Mantido pelo Disease Twin100% com fonte · atualizado 23/09/2026Revisão médica pendente
Informacoes curadas por IA — podem conter imprecisoes

Visão geral

O linfoma primário do sistema nervoso central é uma condição rara caracterizada pelo desenvolvimento de um linfoma não-Hodgkin ou de Hodgkin que surge diretamente no cérebro ou na medula espinhal como lesão primária, sem evidência de comprometimento fora do sistema nervoso central no momento do diagnóstico.[1]

Estima-se que sua prevalência seja de 1 a 9 casos por 1.000.000 de pessoas, com idade de início típica acometendo indivíduos adultos e idosos. O padrão de herança é registrado como não aplicável.[1]

Causas genéticas

Atualmente, não há símbolos de genes específicos associados diretamente à doença na base avaliada, e o padrão de herança não se aplica a esta condição.[1][2]

Diagnóstico

Para a investigação diagnóstica, constam 25 testes genéticos catalogados nos registros de avaliação genética para a condição.[1][2]

Tratamento e manejo

No âmbito do Sistema Único de Saúde (SUS), o manejo encontra-se vinculado ao Protocolo Clínico e Diretrizes Terapêuticas (PCDT) de Linfoma Difuso de Grandes Células B, estabelecido pela Portaria SAS/MS nº 956, de 26 de setembro de 2014, prevendo dispensação conforme autonomia do serviço.[3]

Fontes numeradas em Referências.

Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.

💬
EM LINGUAGEM SIMPLES

O linfoma primário do sistema nervoso central é um tumor raro que se desenvolve originalmente dentro do cérebro ou da medula espinhal, sem afetar outros órgãos no início. A doença se manifesta principalmente em pessoas adultas e idosas, demandando investigação especializada por exames de imagem e biópsia. No Brasil, o tratamento no SUS segue as diretrizes do protocolo de Linfoma Difuso de Grandes Células B, no qual a assistência é prestada conforme a autonomia de cada serviço de saúde. Embora produtos inovadores como o KYMRIAH possuam aprovação na ANVISA e existam terapias aprovadas nos Estados Unidos, a disponibilidade de novas alternativas continua sob intensa pesquisa clínica internacional.

📋
Informacoes curadas por IA — podem conter imprecisoes

Um linfoma não-Hodgkin ou de Hodgkin que surge no cérebro ou na medula espinhal como uma lesão primária. Não há evidência de linfoma fora do sistema nervoso central no momento do diagnóstico.

Pesquisas ativas
57 ensaios
125 total registrados no ClinicalTrials.gov
Publicações científicas
2.967 artigos
Último publicado: 2026 Apr 16
Medicamentos
3 com registro
KYMRIAH, BREYANZI (LISOCABTAGENE MARALEUCEL), KYMRIAH (TISAGENLECLEUCEL)

Tem tratamento?

✓ 3 medicamentos específicos desta doença (registro ANVISA, FDA ou SUS/CEAF)
Ver detalhes, fases e interações →
KYMRIAHBREYANZI (LISOCABTAGENE MARALEUCEL)KYMRIAH (TISAGENLECLEUCEL)

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.44
United States
Início
Adult
+ elderly
🏥
SUS: Cobertura mínimaScore: 0%
CID-10: C83.3
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.967PubMed
Últimos 10 anos200publicações
Pico2025147 papers
Linha do tempo
2026Hoje · 2026🧪 2001Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
✓Aprovado2
3Fase 38
2Fase 255
1Fase 118
·Pré-clínico17
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 100 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Linfoma primário do sistema nervoso central

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Onde estão os ensaios

Com ensaio aberto em 19 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

42 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

NCT07104032 · IGNITE: Study of Tirabrutinib vs Rituximab/Temozolomide for …Recrutando
PHASE3🇧🇷 Brasil · 🇦🇺 Austrália · 🇨🇿 Tchéquia +4
NCT07842835 · Registry of an Integrated Longitudinal Multimodal Biospecime…Recrutando
🇨🇳 China
NCT07839247 · Zanubrutinib Plus Lenalidomide for Frail Elderly Patients Wi…Recrutando
PHASE2🇨🇳 China
NCT07749365 · Hypofractionated Radiotherapy Plus GM-CSF, Pomalidomide and …Recrutando
PHASE2🇨🇳 China
NCT07523737 · Pomalidomide, Anti-PD-1 Antibody Combined With Selinexor (PP…Recrutando
PHASE2🇨🇳 China
NCT07410520 · PD-1 Inhibitor Combined With Rituximab, Methotrexate, and Or…Recrutando
PHASE2🇨🇳 China
NCT07851363 · Rapid Diagnosis and Treatment Response Monitoring of Primary…Recrutando
🇨🇳 China
NCT07350850 · A Multicenter, Prospective Clinical Trial With a Concurrent …Recrutando
PHASE2🇨🇳 China
NCT07062627 · Anbalcabtagene Autoleucel in Relapsed/Refractory CNS Lymphom…Recrutando
PHASE2South Korea
NCT07185373 · Orelabrutinib Combined With Teniposide, Rituximab and Methot…Recrutando
PHASE2, PHASE3🇨🇳 China
NCT06931652 · Study of Epcoritamab in R/R Primary Diffuse Large B-cell Lym…Recrutando
PHASE2🇫🇷 França
NCT07082868 · A Study of Epcoritamab and Ibrutinib in People With Central …Recrutando
PHASE1🇺🇸 Estados Unidos
NCT07188077 · Metabolic and Immunomic Differential Analysis of Primary Cen…Recrutando
🇨🇳 China
NCT06832267 · Rituximab-Methotrexate-Temozolomide-Thiotepa (RMTT) Regimen …Recrutando
NA🇨🇳 China
NCT07074470 · Combination Regimen of Teniposide, PD-1 Monoclonal Antibody …Recrutando
NA🇨🇳 China
NCT06737250 · NB02 (Poseltinib) Combined Rituximab and Lenalidomide in R/R…Recrutando
PHASE2South Korea
NCT06475235 · Pembrolizumab + Chemotherapy in Newly Diagnosed PCNSLRecrutando
PHASE1🇺🇸 Estados Unidos
NCT06556199 · A Study of Selinexor in Combination With Temozolomide and An…Recrutando
PHASE1, PHASE2🇨🇳 China
NCT06554561 · A Study of Pomadomide in Combination With Rituximab and Meth…Recrutando
PHASE1, PHASE2🇨🇳 China
NCT05681195 · Zanubrutinib With Pemetrexed to Treat Relapsed/Refractory Pr…Recrutando
PHASE2🇺🇸 Estados Unidos
NCT06175000 · Maintenance Obinutuzumab in Treating Patients With Central N…Recrutando
PHASE2🇺🇸 Estados Unidos
NCT06940791 · Tirabrutinib Maintenance Versus Placebo in Patients With Pri…Recrutando
PHASE2🇯🇵 Japão
NCT06830421 · Adjusted High-dose Chemotherapy With Autologous Stem Cell Tr…Recrutando
PHASE3🇩🇪 Alemanha
NCT06946407 · Rituximab, Methotrexate, and Tepadina Induction Followed by …Recrutando
NA🇨🇳 China
NCT05931328 · The Study of the Combination of Thiotepa and Pomalidomide fo…Recrutando
PHASE2🇨🇳 China
NCT05600660 · Orelabrutinib,Rituximab and Methotrexate in Newly Diagnosed …Recrutando
PHASE2🇨🇳 China
NCT05351593 · Tafasitamab Plus Lenalidomide in Relapsed CNS LymphomaRecrutando
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT05518383 · B-cell Mature Non-Hodgkin's Lymphoma Treatment Protocol in C…Recrutando
PHASE4Russia
NCT05131022 · A Study of NX-5948 in Adults With Relapsed/Refractory B-cell…Recrutando
PHASE1🇫🇷 França · 🇮🇹 Itália · 🇳🇱 Países Baixos +5
NCT07014943 · High-Dose Gemcitabine, Busulfan, and Thiotepa Followed by AS…Recrutando
PHASE2🇨🇳 China
NCT04656431 · Feasibility of Acquiring Hyperpolarized Imaging in Patients …Recrutando
PHASE1🇺🇸 Estados Unidos
NCT04906096 · Paxalisib (GDC-0084) In Recurrent Or Refractory PCNSLRecrutando
PHASE2🇺🇸 Estados Unidos
NCT04830137 · A Study of NX-2127 in Adults With Relapsed/Refractory B-cell…Recrutando
PHASE1🇺🇸 Estados Unidos
NCT04737889 · Rituximab, Lenalidomide Combined With Methotrexate and Temoz…Recrutando
PHASE2🇨🇳 China
NCT04627753 · Study of Lenalidomide/Rituximab Maintenance for Transplantat…Recrutando
PHASE2South Korea
NCT05036564 · Diagnosis; Objective RespOnse; THErApyRecrutando
NA🇮🇹 Itália
NCT04421560 · Pembrolizumab, Ibrutinib and Rituximab in PCNSLRecrutando
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT04070040 · Camrelizumab for Patients with Recurrent Primary Central Ner…Recrutando
PHASE2🇨🇳 China
NCT04006561 · Prospective Neurobehavioral Functions in Newly-diagnosed Pat…Recrutando
🇹🇼 Taiwan
NCT03328078 · CA-4948-101: Open-Label, Dose Escalation and Expansion Trial…Recrutando
PHASE1, PHASE2🇨🇿 Tchéquia · 🇫🇷 França · 🇮🇱 Israel +4
NCT02657785 · Treatment of PCNSL With R-IDARAM and Intrathecal Immunochemo…Recrutando
PHASE2, PHASE3🇨🇳 China
NCT02836158 · Therapeutic Effects of R-IDARAM and Intrathecal Immunochemot…Recrutando
PHASE2, PHASE3🇨🇳 China

Outros ensaios clínicos

125 ensaios clínicos encontrados, 57 ativos.

Distribuição por fase
NCT07710131 · Neurocognitive Assessment CNSL and LGGEm breve
NA
NCT07764601 · R-MA-PD-1 Versus R-MA in Treatment-naive Primary CNS Lymphom…Em breve
NA🇨🇳 China
NCT07406230 · Methotrexate Early Toxicity MonitoringEm breve
NA🇫🇷 França
NCT07198464 · Relmacabtagene Autoleucel Combined With Autologous Hematopoi…Em breve
NA
NCT06646211 · High-dose Methotrexate Combined with Thiotepa and Zanubrutin…Em breve
PHASE2🇨🇳 China
NCT05901324 · Efficacy of Lenalidomide Maintenance in Primary Central Nerv…Em breve
PHASE2
NCT05347641 · Penpulimab Combined With RMA Treatment of Primary Diagnosis …Em breve
PHASE2
NCT06541665 · Phase I Study Evaluating Tolerability, Safety, Pharmacokinet…Ativo
PHASE1🇯🇵 Japão
NCT05485753 · A Study of GNC-038, a Tetra-specific Antibody, in Patients W…Ativo
PHASE1, PHASE2🇨🇳 China
NCT04947319 · Study of Tirabrutinib (ONO-4059) in Patients With Primary Ce…Ativo
PHASE2🇺🇸 Estados Unidos
NCT04931368 · OptiMATe: De-escalated Induction Treatment in Primary CNS Ly…Ativo
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NCT04462328 · Acalabrutinib and Durvalumab in Primary and Secondary Centra…Ativo
PHASE1🇺🇸 Estados Unidos
NCT04443829 · Immunotherapy Using CAR T-cells to Target CD19 for Relapsed/…Ativo
PHASE1🇬🇧 Reino Unido
NCT04464200 · 19(T2)28z1xx Chimeric Antigen Receptor (CAR) T Cells in Peop…Ativo
PHASE1🇺🇸 Estados Unidos
NCT02203526 · Phase 1 Study of Ibrutinib and Immuno-Chemotherapy Using Tem…Ativo
PHASE1🇺🇸 Estados Unidos
NCT07198087 · A Study to Investigate the Pharmacokinetics of Tirabrutinib …Concluído
PHASE1🇺🇸 Estados Unidos
NCT06714370 · Real-World Evidence of Effectiveness and Safety of Tirabruti…Concluído
🇹🇼 Taiwan
NCT05390814 · [18F]-Fludarabine PET/MRI in Primary Central Nervous System …Concluído
PHASE1🇫🇷 França
NCT06115824 · New-diagnosed PCNSL Treated With Methotrexate (MTX) and Orel…Concluído
🇨🇳 China
NCT06454266 · First-line Treatment of PCNSL With the Combination of Orelab…Concluído
PHASE2🇨🇳 China
NCT05036577 · A Dose-escalating Pilot Study of Orelabrutinib for Newly-dia…Concluído
PHASE1🇨🇳 China
NCT04688151 · Rituximab, Acalabrutinib, and Durvalumab (RAD) in Primary CN…Concluído
PHASE1🇹🇼 Taiwan
NCT04401774 · Nivolumab Maintenance in Newly Diagnosed PCNSL With Persiste…Concluído
PHASE2🇺🇸 Estados Unidos
NCT04934579 · Rituximab,Methotrexate and Lenalidomide in Newly Diagnosed P…Concluído
PHASE2🇨🇳 China
NCT03495960 · Study on Tailored Treatment in Elderly Patients With Newly D…Concluído
PHASE2🇩🇰 Dinamarca · 🇫🇮 Finlândia · 🇮🇹 Itália +1
NCT03703167 · Ibrutinib With Rituximab and Lenalidomide for Patients With …Concluído
PHASE1🇺🇸 Estados Unidos
NCT04134117 · Tisagenlecleucel In Primary CNS LymphomaConcluído
PHASE1🇺🇸 Estados Unidos
NCT03581942 · Copanlisib With Ibrutinib for Patients With Recurrent/ Refra…Concluído
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT03255018 · Pembrolizumab in Relapsed and Refractory Gray-Zone Lymphoma …Concluído
PHASE2🇺🇸 Estados Unidos
NCT05274139 · Treatment of Primary CNS Lymphoma ( FTD )Concluído
PHASE2🇨🇳 China
NCT02669511 · PQR309 in Patients With Relapsed or Refractory Primary Centr…Concluído
PHASE2
NCT02315326 · Bruton's Tyrosine Kinase (BTK) Inhibitor, Ibrutinib, in Pati…Concluído
PHASE1, PHASE2🇺🇸 Estados Unidos
NCT02301364 · Buparlisib (BKM120) In Patients With Recurrent/Refractory Pr…Concluído
PHASE2🇺🇸 Estados Unidos
NCT01647971 · Study of the Efficacy and Safety of Ublituximab in Patients …Concluído
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NCT01421524 · Study of CC-122 to Evaluate the Safety, Tolerability, and Ef…Concluído
PHASE1🇧🇪 Bélgica · 🇫🇷 França · 🇮🇹 Itália +2
NCT00267865 · Chemotherapy and HAART to Treat AIDS-related Primary Brain L…Concluído
PHASE2🇺🇸 Estados Unidos
NCT00193973 · Idarubicin Based Combined Modality Therapy in Primary CNS Ly…Concluído
PHASE2🇦🇺 Austrália · New Zealand
NCT06445257 · A Clinical Study Evaluating the Safety and Efficacy of ZRMT …UNKNOWN
NA🇨🇳 China
NCT05896007 · Zanubrutinib Combined With Rituximab and Chemotherapy for Ne…UNKNOWN
PHASE2🇨🇳 China
NCT05135858 · Dose Dense Re-challenge of High Dose Methotrexate With Gluca…UNKNOWN
PHASE1🇫🇷 França
NCT04845139 · A Case Study of Nivolumab for Relapsed/Refractory Primary Ce…UNKNOWN
NA🇨🇳 China
NCT05549284 · Orelabrutinib,Rituximab and Methotrexate in Newly-diagnosed …UNKNOWN
PHASE2🇨🇳 China
NCT05390749 · Sequential Treatment With RO-MTX After Pomalidomide, Orelabr…UNKNOWN
PHASE2🇨🇳 China
NCT05334238 · Orelabrutinib Maintenance Therapy After ASCT in Primary Cent…UNKNOWN
PHASE3🇨🇳 China
NCT05021770 · Orelabrutinib in Combination With Thiotepa in Refractory and…UNKNOWN
PHASE1, PHASE2🇨🇳 China
NCT05425654 · RL-MPV Followed by BBC HCT Using Autologous Stem Cells and M…UNKNOWN
PHASE2Russia
NCT04831658 · A Prospective Clinical Study of BTK Inhibitor, PD-1 and Form…UNKNOWN
PHASE1, PHASE2🇨🇳 China
NCT04899427 · Phase II Study of Orelabrutinib Combined With PD-1 Inhibitor…UNKNOWN
PHASE2🇨🇳 China
NCT04457869 · A Study of F520 in Relapsed/Refractory Primary Central Nervo…UNKNOWN
PHASE2
NCT04514393 · Ibrutinib With Methotrexate and Temozolomide for Patients Wi…UNKNOWN
PHASE2🇨🇳 China
NCT04481815 · Rituximab Plus Methotrexate With or Without Lenalidomide in …UNKNOWN
PHASE2🇨🇳 China
NCT04253496 · Mathematic Modeling at Micro and Macroscopic Level of Primar…UNKNOWN
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NCT04066920 · IBER Salvage Treatment Followed by Ibrutinib Maintenance for…UNKNOWN
PHASE2South Korea
NCT04083066 · Open Randomized Prospective Clinical Study of R-FPD Versus R…UNKNOWN
PHASE4🇨🇳 China
NCT04120350 · R2-MTX With Lenalidomide Maintenance in Newly-diagnosed PCNS…UNKNOWN
PHASE1, PHASE2🇨🇳 China
NCT04438044 · A Study to Evaluate ICP-022 in Patients With R/R PCNSL and S…UNKNOWN
PHASE2🇨🇳 China
NCT03569995 · [CREMA]Combination of R-M Followed by R-A in Elderly Patient…UNKNOWN
PHASE2South Korea
NCT03733327 · BUCYE Conditioning Regimen for PCNSL Undergoing Auto-HSCTUNKNOWN
PHASE2, PHASE3🇨🇳 China
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
1.864 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.864

#1

The Regulation of Vasomotor and Cardiorespiratory Pulsations Is Disrupted in Primary Central Nervous System Lymphoma: A Case-Control fMRI Study.

Human brain mapping2026 Mar

Primary central nervous system lymphoma (PCNSL) alters (peri)vascular structures while increasing vasomotor and cardiorespiratory pulsations within the brain. Vasomotor pulsations may arise from amplitude modulations of respiratory (RPE) and cardiovascular (CHE) pulsations while cardiovascular fluctuations may be modulated by respiration through cardiorespiratory amplitude modulation (CREM). In this study, we examined glymphatic cerebrospinal fluid convection in brains of PCNSL patients by assessing these waves. Thirty PCNSL patients (median 66y; 9 females) and 40 healthy age-matched controls (median 62y; 29 females) were scanned using an fMRI-based MREGBOLD sequence. Respective MREGBOLD fluctuation amplitudes (AFRPE; AFCHE; AFCREM) were compared between groups using nonparametric permutation. Regional amplitudes were compared using Mann-Whitney analysis and Cox survival analysis. Subject-specific pulsations were analyzed through Z-score mapping. AFCREM and AFRPE were significantly elevated across PCNSL brains, with lesser increases in AFCHE. However, only significant increases in AFRPE remained after correcting for sex and head displacement. AFRPE showed a link to mortality as it was markedly elevated in deceased patients. While elevations in all pulsations were present within (peri)tumoral regions, AFRPE elevations extended into extra-tumoral white matter and grey matter. Thus, altered cardiorespiratory fluctuations give rise to dysfunctional vasomotor and CSF pulsations in PCNSL, predicting impaired glymphatic function.

#2

KPT-8602 combined with IFN-γ released ZBP1-PANoptosome to inhibit the progression of primary central nervous system lymphoma.

Experimental cell research2026 Apr 15

To investigate whether the combination of KPT-8602 and interferon-γ (IFN-γ) can inhibit the progression of primary central nervous system lymphoma (PCNSL) by activating ZBP1-mediated PANoptosis. Human lymphoma cell lines including Raji (Burkitt's lymphoma) and U-2932 (Diffuse large B-cell lymphoma, DLBCL) were employed. The synergistic induction of PANoptosis by KPT-8602 and IFN-γ was evaluated using CCK8 assay, flow cytometry, YO-PRO/PI staining, and Western blot analysis. A mouse model of PCNSL was established through stereotaxic brain implantation. The efficacy and molecular mechanisms of the combined therapy were evaluated using HE staining, TUNEL staining, ELISA, multiplex immunofluorescence, and Western blot analysis. The combination therapy of KPT-8602 and IFN-γ exhibited synergistic inhibitory effects on lymphoma cell growth (CI < 1). The combination therapy group significantly enhanced apoptosis and PANoptosis, as well as upregulated the mRNA levels of PANoptosis-related inflammatory factors (IL-18, IL-1β) and the expression of PANoptosis-associated proteins. In the nude mouse model, the tumor volume was significantly smaller in the combination therapy group compared to the single-drug group. Additionally, there was a notable increase of TUNEL staining positivity in tumor tissue and elevated levels of the inflammatory factors IL-6 and CXCL13 in the cerebrospinal fluid. The Western blot analysis of tumor tissue was consistent with in vitro experiments, demonstrating significant activation of the PANoptosis pathway. The combination of KPT-8602 and IFN-γ can activate the pan-apoptotic pathway by upregulating ZBP1, thereby effectively inhibiting the growth of PCNSL. This study presented a promising new combination treatment strategy for PCNSL.

#3

A case report: primary central nervous system lymphoma misdiagnosed as neuromyelitis optica.

BMC neurology2026 Feb 16

Primary central nervous system lymphoma (PCNSL) occurring in the spinal cord is very rare. Due to its atypical clinical symptoms and imaging manifestations, it is extremely easy to be misdiagnosed as other diseases. This study reports a case of PCNSL misdiagnosed as neuromyelitis optica (NMO) and analyses neuroimaging characteristics that may aid in the diagnostic evaluation of PCNSL. The case reports a 44-year-old man who presented with clinical symptoms including persistent neck pain, upper limb weakness, blurred vision in the left eye. His Aquaporin4 antibody (AQP4 antibody) was slightly positive (1:10), and cervical spinal cord magnetic resonance imaging (MRI) showed the continuous segment and multifocal areas throughout cervical spinal cord with obvious homogenous enhancement, leading to an initial misdiagnosis of NMO. His symptoms progressively improved after steroid pulse treatment, but worse after steroid tapering. Following magnetic resonance spectroscopy (MRS) and positron emission tomography (PET) examinations, PCNSL should be firstly considered. Immunohistochemical analysis confirmed this diagnosis. After undergoing chemotherapy, the patient’s lesion area was reduced. Unfortunately, the patient ultimately died after being hospitalized for 9 months. Since PCNSL, especially in the spinal cord, is uncommon, its neuroimaging appearance could be misdiagnosed with other conditions, such as NMO, when there is positive expression of AQP4 antibody. Special attention should be made to the clinical symptoms and follow-up brain and spinal MRI as well as histopathologic examination are recommended for early diagnosis and treatment.

#4

Penpulimab combined with rituximab, high-dose methotrexate, and cytarabine (Pen-RMA) in newly diagnosed primary central nervous system lymphoma (PCNSL): a phase 2 trial.

Blood cancer journal2026 Feb 11

This clinical trial (NCT05347641) evaluated efficacy and safety of penpulimab combined with rituximab, high-dose methotrexate, and cytarabine (Pen-RMA) in patients with newly diagnosed (ND) primary central nervous system lymphoma (PCNSL). Patients received an induction treatment of six cycles of Pen-RMA every 3 weeks. Patients younger than 60 years who achieved at least partial remission (PR) underwent autologous stem cell transplantation (ASCT), followed by 8 cycles of penpulimab as maintenance therapy. For patients over 60 years or not eligible for ASCT who achieved complete remission (CR) after induction therapy received 8 cycles of penpulimab as maintenance treatment, and those who achieved PR were treated with whole brain radiotherapy (WBRT) combined with 8 cycles of penpulimab. Patients who achieved stable disease (SD) or progressive disease (PD) were withdrawn from this study. The primary endpoint was 2-year progression-free survival (PFS). Between April 2022 and December 2023, twenty-six ND-PCNSL patients were enrolled, and 23 patients were included in the intention-to-treat analysis. The median age was 65 (38‒74) yr. The overall response rate (ORR) and CR rate after the induction therapy were 95.7% and 91.3%, respectively. At a median follow-up of 29.4 months, the median PFS and overall survival (OS) were not reached. 2-year PFS and OS were 70.7% and 75.0%, respectively. The most frequent treatment-related adverse events were leukopenia, anemia, neutropenia, and thrombocytopenia. Grade 3 or worse treatment-related adverse events occurred in 7 (30.4%) of 23 patients. Cerebrospinal fluid (CSF) circulating-tumor DNA (ctDNA) monitoring was performed in 13 patients with imaging CR and 1 with PR after induction treatment. Among them, 8 had CSF ctDNA clearance while 6 were positive. Overall, Pen-RMA regimen demonstrated encouraging antitumor activity with a manageable toxicity in PCNSL.

#5

Primary central nervous system lymphoma: Evolving treatment strategies to achieve improved long-term disease control.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners2026 Mar 17

ObjectiveDespite high initial response rates to chemotherapy, long-term disease control in primary central nervous system lymphoma (PCNSL) remains challenging, with high relapse rates and no universally accepted standard for induction or consolidation therapy. This review discusses current strategies, new advancements and clinical trial results for PCNSL.Data SourcesAn extensive Medline, Embase and Cochrane search of peer-reviewed sources reporting on pharmacotherapy of PCNSL was performed (1/1/2000-12/31/2025). Analysis of original research including clinical trial results, retrospective series and other reports were included in this review.Data SummaryThis review summarizes the current understanding of PCNSL, including epidemiology, clinical presentation, diagnostic evaluation, and response assessment, with a focus on pharmacologic management. We discuss modern induction strategies centered on high-dose methotrexate (HD-MTX) and rituximab, consolidation approaches including reduced-dose whole-brain radiotherapy (WBRT), autologous stem cell transplantation (ASCT), and maintenance strategies, as well as emerging therapies for relapsed or refractory disease. A case of a 76-year-old patient achieving sustained remission exceeding seven years following rituximab plus HD-MTX induction and consolidative therapy is presented to illustrate the potential for durable disease control and even cure. These data underscore the importance of individualized treatment selection based on patient age, fitness level and response to induction and highlight that lasting remission is achievable in select patients with optimized, multimodal therapy.ConclusionsDespite its rarity, PCNSL remains a therapeutically challenging malignancy. HD-MTX-based chemotherapy remains the cornerstone of first-line treatment, and the addition of rituximab has been associated with more sustained responses. Although relapses are not uncommon, long-term remission is achievable. Clinical trials of newer agents, alone or added to a backbone of traditional chemotherapy, are warranted in the 1st and subsequent lines of therapy. Continued prospective research and international collaboration are essential to integrate precision oncology to the bedside of patients with PCNSL.

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2026

Secondary graft failure after autologous stem cell transplantation for primary central nervous system lymphoma responds to ciclosporin and eltrombopag.

Leukemia &amp; lymphoma
2026

Primary central nervous system lymphoma: Evolving treatment strategies to achieve improved long-term disease control.

Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners
2026

Radiomics-based differentiation between glioblastoma and primary central nervous system lymphoma: CT vs MRI.

Cancer imaging : the official publication of the International Cancer Imaging Society
2026

Cystatin C regulates cell division in primary central nervous system lymphoma.

Scientific reports
2026

Treatment initiation by positive liquid biopsy alone in primary central nervous system lymphoma: A retrospective analysis of a multi-institutional study.

Neuro-oncology advances
2026

The Regulation of Vasomotor and Cardiorespiratory Pulsations Is Disrupted in Primary Central Nervous System Lymphoma: A Case-Control fMRI Study.

Human brain mapping
2026

Fotemustine-containing chemotherapy suggests lower toxicity with comparable efficacy versus high-dose methotrexate-based regimens: a retrospective cohort analysis.

Therapeutic advances in medical oncology
2026

Effective high-dose methotrexate toxicity reversal using fixed-dose glucarpidase in obese patients: a case series.

Journal of medical case reports
2026

HIV-associated lymphoproliferative disorders: Single-center experience.

Medicine
2026

Improving MRI-based differentiation of brain metastasis, glioblastoma, and primary CNS lymphoma using deep learning.

Neuroradiology
2026

Movement disorders due to primary central nervous system lymphoma: An illustrative case and systematic literature review.

Revue neurologique
2026

Real-world comparison of BTK inhibitors and lenalidomide as first-line maintenance in primary CNS lymphoma: a multi-center retrospective study.

Journal of neuro-oncology
2026

An Update on Treatments for Recurrent Primary Central Nervous System Lymphoma.

Current oncology reports
2026

Primary Dural Diffuse Large B-Cell Lymphoma: A Report of a Rare Case and Review of the Literature.

Cureus
2026

Superiority of high-dose cytarabine-based mobilisation over cyclophosphamide or plerixafor in primary CNS lymphoma.

Annals of hematology
2026

KPT-8602 combined with IFN-γ released ZBP1-PANoptosome to inhibit the progression of primary central nervous system lymphoma.

Experimental cell research
2026

Primary Central Nervous System Lymphoma With Testicular and Prostatic Involvement: A Case Report.

Cureus
2026

Brain Lesions Associated with Human Immunodeficiency Virus Infection in the Antiretroviral Therapy Era: A Single-center Experience in Japan.

Neurologia medico-chirurgica
2026

Advances in liquid diagnostics for primary central nervous system lymphoma: a narrative review.

Neurologia i neurochirurgia polska
2026

A case report: primary central nervous system lymphoma misdiagnosed as neuromyelitis optica.

BMC neurology
2026

[Coexistence of primary central nervous system lymphoma and cerebral toxoplasmosis in a patient with advanced HIV infection and negative serology].

Revista espanola de quimioterapia : publicacion oficial de la Sociedad Espanola de Quimioterapia
2026

Time matters: The prognostic impact of diagnostic delay on survival in primary central nervous system lymphoma-a single-center, retrospective real-world study.

Neuro-oncology advances
2026

Exosomal ANGPTL2 in cerebrospinal fluid as a novel prognostic biomarker for primary central nervous system lymphoma.

Frontiers in immunology
2026

Prognostic Assessment of Surgical Strategies and Extent of Resection in Methotrexate-Based Treatment for Primary Central Nervous System Lymphoma: A Propensity-Matched Analysis.

World neurosurgery
2026

Consolidation reduced-dose whole-brain radiation therapy with response-adapted boost in older patients with newly diagnosed primary central nervous system lymphoma.

Journal of neuro-oncology
2026

Clinical Characteristics and Prognosis of Primary Central Nervous System Lymphoma: A Retrospective Analysis.

Cancers
2026

Strategies for Managing Toxicities With High-Dose Methotrexate in Haematological Malignancies: Lessons From Clinical Cases.

Clinical case reports
2026

Penpulimab combined with rituximab, high-dose methotrexate, and cytarabine (Pen-RMA) in newly diagnosed primary central nervous system lymphoma (PCNSL): a phase 2 trial.

Blood cancer journal
2026

A 62-Year-Old-Man With Severe Daytime Sleepiness After Primary Central Nervous System Lymphoma.

Chest
2026

Use of Glucarpidase for Methotrexate-Induced Nephrotoxicity During the Treatment of Primary Central Nervous System Lymphoma.

Cureus
2026

Incidence and Management of Tirabrutinib-associated Cutaneous Adverse Events: A Case Series.

Yakugaku zasshi : Journal of the Pharmaceutical Society of Japan
2026

Composite lymphoma comprising adult T-cell leukemia/lymphoma and Epstein-Barr virus-positive primary central nervous system lymphoma.

Journal of clinical and experimental hematopathology : JCEH
2026

Zanubrutinib, lenalidomide, rituximab, temozolomide ± methotrexate as first-line treatment for primary central nervous system lymphoma: A prospective, open-label, multicentre clinical trial.

British journal of haematology
2026

Diagnostic utility of cerebrospinal fluid IgG index, 24-hour IgG synthesis rate, and immunophenotyping in primary central nervous system large B-cell lymphoma: A case report.

Medicine
2026

Differentiation of high-grade glioma and primary central nervous system lymphoma based on imaging heterogeneity scoring system.

Neurosurgical review
2025

Development and validation of multiple machine learning algorithms for differentiating primary central nervous system lymphoma from adult-type diffuse glioma: an interpretable and multicenter study.

Frontiers in oncology
2026

Primary dural lymphoma of the chronic lymphocytic lymphoma/small lymphocytic lymphoma-mimicking meningioma: illustrative case.

Journal of neurosurgery. Case lessons
2026

Ibrutinib in combination with rituximab, methotrexate, vincristine, and procarbazine (R-MVP/i) for newly diagnosed primary CNS lymphoma (PCNSL).

Neuro-oncology
2026

Induction chemotherapy with methotrexate, nimustine, and procarbazine for primary CNS lymphoma in the elderly: a retrospective evaluation of safety and efficacy.

Journal of neuro-oncology
2026

Early Diagnostic Value of Contrast-Enhanced MRI in Distinguishing Toxoplasmic Encephalitis from CNS Lymphoma: A Case Report of AIDS with Seronegative Toxoplasmosis.

Journal of the International Association of Providers of AIDS Care
2026

Educational and regional differences in population-based incidence and survival of primary central nervous system lymphoma in Finland.

BMC cancer
2026

Stereotactic Radiosurgery for Primary Central Nervous System Lymphoma: Results From the International Radiosurgery Research Foundation.

Neurosurgery
2025

Behavioral Changes as the Initial Presentation of Primary Central Nervous System Lymphoma: A Case Report.

Cureus
2025

It Is Not Always Metastasis: A Case Report of a Primary Nervous System Anaplastic Large Cell Lymphoma and the Relevance of Brain Biopsy.

Cureus
2025

Primary central nervous system lymphoma in children: Insights from the three-year experience at the largest public-sector pediatric oncology center in Pakistan.

Pakistan journal of medical sciences
2025

High-Dose Methotrexate at All Ages: Safety, Efficacy, and Outcomes from the HDMTX European Registry.

Cancers
2026

Multifocal anaplastic astrocytoma mimicking primary central nervous system lymphoma: A case report.

Medicine
2026

A first-line regimen combining Bruton's tyrosine kinase and programmed cell death protein-1 inhibitors with chemotherapy excluding methotrexate achieves high response rates in primary central nervous system lymphoma.

Cancer
2025

LINNDA: Lymphoma identification through neural network detection aid.

iScience
2025

New biomarkers for the diagnosis of primary central nervous system lymphoma in CSF: A multicenter retrospective cohort study.

Neuro-oncology advances
2025

Evaluation of programmed cell death ligand-1 expression in primary central nervous system lymphoma using whole-tumor histogram analysis of multiparametric MRI: implications for immunotherapy selection.

Frontiers in immunology
2025

Primary Central Nervous System Lymphoma: A Case Report of the Neurological Great Mimicker.

Cureus
2025

A Durable Remission Following Pseudo-Progression in Tirabrutinib Treatment for Relapsed Primary Central Nervous System Lymphoma: A Case Study.

Case reports in hematology
2026

Prognostic impact of procarbazine in R-MPV followed by reduced-dose whole-brain radiotherapy and cytarabine for primary central nervous system lymphoma.

Leukemia &amp; lymphoma
2025

Successful combined treatment for primary central nervous system lymphoma with massive hemorrhage: Illustrative case.

Surgical neurology international
2025

[Autologous hematopoietic stem cell transplantation with TBE conditioning in patients with primary central nervous system lymphoma].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Real-world survival, cognitive, and quality of life outcomes of methotrexate/ifosfamide plus whole brain radiotherapy for newly diagnosed primary central nervous system lymphoma.

Therapeutic advances in hematology
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Intraocular Lymphoma: A Review.

Journal of ophthalmic &amp; vision research
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A retrospective analysis of autologous stem cell transplantation conditioning with reduced-dose busulfan/thiotepa for patients with central nervous system lymphomas at a single institution.

International journal of hematology
2025

Tirabrutinib rechallenge achieved complete response for recurrent primary central nervous system lymphoma: illustrative case.

International cancer conference journal
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Successful treatment of primary intramedullary spinal cord lymphoma based on the molecular diagnosis of a MYD88 mutation in the cerebrospinal fluid: a case report.

International cancer conference journal
2025

Phase II Trial of an Orelabrutinib-Based Combination Therapy in Newly Diagnosed Primary Central Nervous System Lymphoma.

Blood and lymphatic cancer : targets and therapy
2025

Effectiveness and safety of orelabrutinib combined with rituximab, temozolomide, methotrexate, and cytarabine in intensive chemotherapy-unfit patients with PCNSL in China.

European journal of medical research
2025

Prebiopsy Steroids and Diagnostic Yield in Patients With Diffuse Large B-Cell Lymphoma.

JAMA network open
2025

Role of Geriatric Assessment Scores as Predictors of Intensive Therapy Feasibility and Survival in Elderly Patients with Primary CNS Lymphoma.

Cancers
2026

Multiple primary central nervous system lymphoma in the ventricular system and interpeduncular cistern: A case report.

Oncology letters
2025

A Case Report of HIV-Associated Pituitary Lymphoma and Review of the Literature.

Clinical case reports
2025

Development and validation of a convolutional neural network for automatic differentiation of primary central nervous system lymphoma and glioblastoma.

Cancer imaging : the official publication of the International Cancer Imaging Society
2025

A Novel Prognostic Model for Primary CNS Lymphoma Incorporating Clinico-Laboratory Parameters.

Neuro-oncology
2025

Elevated polyglutamylation and tau phosphorylation levels are associated with cognitive impairment at diagnosis in patients with primary central nervous system lymphoma.

Alzheimer's research &amp; therapy
2025

[Primary lymphoma of the central nervous system].

Arkhiv patologii
2026

Outcomes of de novo primary central nervous system lymphoma using the MATRix regimen for induction followed by thiotepa-based conditioning: a GELTAMO analysis.

Bone marrow transplantation
2025

Length of hospital stay and outcome in primary central nervous system lymphoma: a French retrospective multicentric study.

Annals of hematology
2025

Efficacy and Limitations of Flow Cytometry for the Rapid Diagnosis of Primary Central Nervous System Lymphoma.

Cancers
2025

Genomic landscape and molecular subtypes of primary central nervous system lymphoma.

Blood cancer journal
2025

Diagnostic Potential of Cerebrospinal Fluid Biomarkers in Diagnosis of Primary Central Nervous System Lymphoma: A Systematic Review and Meta-Analysis.

Annals of Indian Academy of Neurology
2025

Electron Density and Effective Atomic Number as Quantitative Biomarkers for Differentiating Malignant Brain Tumors: An Exploratory Study with Machine Learning.

Tomography (Ann Arbor, Mich.)
2025

Outcomes for Primary Central Nervous System Lymphoma from a Single Institution.

Hematology reports
2025

Primary Central Nervous System Lymphoma Associated With Immune Deficiency and Dysregulation: Challenges in Treatment and Management.

Cureus
2025

A Case of Primary Central Nervous System Lymphoma Mimicking a Demyelinating Disorder: From Steroids to Stem Cell Transplant.

Cureus
2026

Primary central nervous system lymphoma mimicking an otitis media complication: an unusual presentation with sigmoid sinus thrombosis.

European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
2025

Clonal identification and homology differentiate primary central nervous system lymphoma from non-central nervous system lymphoplasmacytic lymphoma: a case report.

Journal of medical case reports
2025

Orelabrutinib plus reduced-dose whole brain radiotherapy in elderly primary central nervous system lymphoma: a case report.

Frontiers in oncology
2025

[The Efficacy and Safety of Modified Thiotepa-Based Conditioning Followed by Autologous Stem Cell Transplantation in Primary CNS Lymphomas].

Zhongguo shi yan xue ye xue za zhi
2025

Hypothalamic-Pituitary Axis Involvement in Primary Central Nervous System Lymphoma: A Systematic Review.

The Journal of clinical endocrinology and metabolism
2025

Chemotherapy-Free Salvage Therapy with Rituximab, Lenalidomide, and Poseltinib in Relapsed or Refractory Primary Central Nervous System Lymphoma: A Multi-Center, Phase II Study.

Cancer research and treatment
2025

Primary Dural Diffuse Large B-Cell Lymphoma: A Case Report.

JMA journal
2025

Role of rituximab in treatment of patients with primary central nervous system lymphoma: An updated systematic review and meta-analysis.

Acta neurologica Belgica
2025

u-LINNDA: A protocol for user-optimized lymphoma identification through neural network detection aid.

STAR protocols
2025

Mass cytometric analysis of circulating immune landscape in primary central nervous system lymphoma.

Frontiers in immunology
2026

Multicenter randomized phase II study of R-MPV-A chemoimmunotherapy with or without low-dose whole-brain radiotherapy for newly-diagnosed primary CNS lymphoma.

Neuro-oncology
2025

Interpretable radiomics-based machine learning model for differentiating glioblastoma from primary central nervous system lymphoma using contrast-enhanced T1-weighted imaging.

Scientific reports
2025

Plain language summary about tirabrutinib in relapsed/refractory primary central nervous system lymphoma.

Future oncology (London, England)
2025

Evaluation of Prognostic Factors in Primary Central Nervous System Lymphomas: A Single-Center Study.

Turkish neurosurgery
2025

Preoperative multiparameter MRI-based prediction of Ki-67 expression in primary central nervous system lymphoma.

Precision radiation oncology
2025

Study of plasma exosomal miRNAs as novel biomarkers for differentiating primary central nervous system lymphoma and glioblastoma.

BMC cancer
2025

Sellar-suprasellar pituitary lymphoma mimicking pituitary adenoma: a case report with literature review.

Journal of surgical case reports
2025

Clinical utility of repeat brain biopsy in recurrent or refractory primary CNS lymphoma - a diagnostic dilemma.

Journal of neuro-oncology
2025

Long-Term Real-World Outcomes of Primary CNS Lymphoma Patients Treated With MATRix Regimen Are Similar to IELSG32 Trial Results.

Hematological oncology
2025

Specific Marker Gene Analysis for Primary Central Nervous System Lymphoma Based on Methylation Difference and Development of Detection Primers.

Brain and behavior
2025

[Clinical characteristics and prognosis of 52 patients with primary central nervous system lymphoma].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2025

Targeting PD-L1 for PCNS-DLBCL: from molecular effects to clinical translation.

Frontiers in immunology
2025

Clinical characteristics and prognosis of ventricular primary central nervous system lymphoma: a case series and literature review.

Chinese neurosurgical journal
2025

Population pharmacokinetics of high-dose methotrexate and 7-hydroxy methotrexate in Chinese adults with primary central nervous system lymphoma.

British journal of haematology
2025

Magnetic Resonance Spectroscopy in the Evaluation of Biopsy-Indeterminate Primary Central Nervous System Lymphoma: A Case Report.

Cureus
2025

Lenalidomide-rituximab with high-dose methotrexate for treatment of patients with newly diagnosed primary cns lymphoma: a promising first-line approach.

Annals of hematology
2025

Corticosteroid Use Before Stereotactic Brain Biopsy for Suspected Lymphoma.

Neurosurgery
2025

Lymphomatosis cerebri presenting with rapidly progressive parkinsonism and Holmes tremor: a case report.

BMC neurology
2025

Uncertainty-aware ensemble of foundation models differentiates glioblastoma from its mimics.

Nature communications
2025

Case Report: Chronic subdural hematoma secondary to primary central nervous system lymphoma.

Frontiers in surgery
2025

The role of surgical resection and stereotactic radiosurgery in PCNS-DLBCL: a retrospective analysis of clinical benefits.

International journal of surgery (London, England)
2025

Automatic Segmentation of Primary Central Nervous System Lymphoma at Clinical Routine Postcontrast T1-weighted MRI.

Radiology. Imaging cancer
2026

A Case of Relapsed/Refractory Primary Central Nervous System B-Cell Lymphoma with Renal Insufficiency Successfully Treated with Linperlisib.

Turkish journal of haematology : official journal of Turkish Society of Haematology
2025

The Landscape of Primary Central Nervous System Lymphoma (PCNSL): Clinicopathologic and Genomic Characteristics and Therapeutic Perspectives.

Cancers
2025

Meta-analysis evaluating the efficacy and safety of various Bruton tyrosine kinase (BTK) inhibitors for central nervous system lymphoma: Novel covalent BTK inhibitors, except for ibrutinib, also demonstrate good efficacy in the treatment of primary central nervous system lymphoma.

Cancer
2025

Case Report: Effective methotrexate removal by combined hemodialysis and polymeric resin hemoadsorption.

Frontiers in nephrology
2025

Diagnostic Performance of CSF Interleukin-10 in Primary Central Nervous System Lymphoma: A Retrospective Study.

Cureus
2025

Co-existence of Primary Glioma and Lymphoma: An Unusual Case Report.

NMC case report journal
2025

Management of primary central nervous system lymphoma and coexisting heart failure with reduced ejection fraction: a narrative review.

Cardio-oncology (London, England)
2025

Radiation Retinopathy After Whole-Brain Radiotherapy in a Patient With Pineal Gland Tumor.

Journal of vitreoretinal diseases
2025

NMDAR-Antibody Encephalitis Diagnosed With Primary Central Nervous System Lymphoma: A Case Series.

Annals of clinical and translational neurology
2026

Bloodstream Infection Caused by Mycobacterium neoaurum in a Primary Central Nervous System Lymphoma Patient with Toxic Epidermal Necrolysis.

Internal medicine (Tokyo, Japan)
2025

Multiparametric MRI for differential diagnosis of primary central nervous system lymphoma and atypical glioblastoma: an analysis incorporating DWI, DCE-MRI, and contrast agent preload DSC-PWI.

BMC medical imaging
2025

The Role of Percentage Signal Recovery (PSR) in MRI Perfusion for the Diagnosis of Primary Central Nervous System Lymphoma.

Cureus
2025

Primary Central Nervous System B-Cell Lymphoma Presenting with Fluctuating Basal Ganglia and Cortical Lesions.

Journal of the Belgian Society of Radiology
2026

Tumor Habitats Based on Multiparametric MRI Distinguish Atypical Glioblastoma From Primary Central Nervous System Lymphoma: Imaging-Pathologic Correlation.

Journal of magnetic resonance imaging : JMRI
2025

Risk factors associated with high-dose methotrexate induced toxicities in primary central nervous system lymphoma.

Frontiers in pharmacology
2025

Primary central nervous system lymphomas in three "blue books" and correlation of recently defined primary large B-cell lymphoma of immune-privileged sites: Single-center experience.

Indian journal of pathology &amp; microbiology
2025

Clinicopathological Significance of Tumor-Infiltrating T Lymphocytes and Macrophages in Primary Large B-Cell Lymphoma of Immune-Privileged Sites.

Cancer research and treatment
2025

Liquid Biopsy and Epigenetic Signatures in AML, ALL, and CNS Tumors: Diagnostic and Monitoring Perspectives.

International journal of molecular sciences
2025

Secondary Versus Recurrent Central Nervous System Lymphoma: Evaluating the Similarities and Differences in a Single-Institution Cohort.

Cureus
2025

Case Report: CAR-T therapy for primary cerebellar ALK-negative anaplastic large cell lymphoma.

Frontiers in immunology
2025

Clinical characteristics and prognosis of primary central nervous system lymphoma in China: a single-center retrospective analysis of 325 patients.

Leukemia &amp; lymphoma
2025

Histogram analysis based on DTI and NODDI for differentiating atypical high-grade glioma from primary central nervous system lymphoma.

Frontiers in neurology
2025

New treatment strategies for primary central nervous system lymphoma.

Current opinion in oncology
2025

Molecular Characterization and Therapeutic Implications of MYD88 and CDKN2A in Primary CNS Lymphoma.

Anticancer research
2025

Prognosticating salvage stereotactic radiosurgery outcomes in relapsed primary central nervous system lymphoma: A machine learning-driven decision tree analysis.

Translational oncology
2025

Exploration of the Optimal Treatment Modality for Vitreoretinal Lymphoma: A PRISMA Compliant Meta-Analysis and Systematic Review.

Cancer medicine
2025

The Diagnostic Yield of Cerebrospinal Fluid Analysis for the Diagnosis of Primary Central Nervous System Lymphoma: A Systematic Review.

Cancers
2025

A nomogram for predicting the diagnosis of central malignant tumors based on preoperative clinical characteristics and laboratory indicators: a diagnostic study.

International journal of surgery (London, England)
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Comparative analysis of the tumor microenvironment in primary CNS and testicular large B-cell lymphomas using digital image analysis and its implications for immunotherapy.

Human pathology
2025

PDL1 Gene Gain Predicts an Unfavorable Prognosis in HIV-Positive Primary Central Nervous System Lymphoma.

Current oncology (Toronto, Ont.)
2025

Case report: diagnostic challenges of primary central nervous system anaplastic large cell lymphoma, ALK-positive.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2025

Programmed death inhibitors in CNS B-cell lymphoma: A literature review.

Blood reviews
2025

Real-World Efficacy and Safety of the Combination of Rituximab, Lenalidomide, and Ibrutinib in Patients with Relapsed and/or Refractory Non-Hodgkin Lymphoma.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2025

Vasomotor fluctuations are increased in primary central nervous system lymphoma: a case-control study with fast functional MRI.

Brain communications
2025

Diagnostic Odyssey: Primary CNS Lymphoma (Lymphoma Cerebri) Presenting as Rapidly Progressive Dementia.

Case reports in hematology
2025

Circulating tumor DNA in B cell lymphomas.

Current opinion in oncology
2025

Clinical Characteristics, Prognostic Risk Factors, and Primary Treatment for Elderly Patients with Primary Central Nervous System Lymphoma: A Seer Database-Based Research.

Blood and lymphatic cancer : targets and therapy
2025

Dexamethasone-associated regression of glioblastoma.

Surgical neurology international
2025

Rituximab, high-dose methotrexate plus orelabrutinib as induction therapy in newly diagnosed primary central nervous system lymphoma.

Leukemia
2025

Clinical Predictors and Recurrence Characteristics Following Radiotherapy for Primary Central Nervous System Lymphoma: A Retrospective Cohort Study.

Cancers
2025

Whole-Eye Radiation for the Local Control of Choroidal Lymphoma in Primary Central Nervous System Lymphoma: A 14-Year Case Study.

Cureus
2025

Ultra-sensitive detection of microRNA in intraocular fluid using optical fiber sensing technology for central nervous system lymphoma diagnosis.

Reports on progress in physics. Physical Society (Great Britain)
2025

Meeting Report From the 2024 Symposium on IRAK4 in Cancer: Highlights and Clinical Updates.

Clinical lymphoma, myeloma &amp; leukemia
2025

Outcomes at a single institution with MTR induction and consolidation in the management of primary CNS lymphoma.

Blood neoplasia
2025

Clinically probable paraneoplastic vasculitic peripheral neuropathy in primary central nervous system lymphoma: a case report.

BMC neurology
2025

Molecular diagnosis of vitreoretinal lymphoma.

Taiwan journal of ophthalmology
2025

Immune Checkpoint Molecule Expression, 9p24.1 Gene Alterations, and Tumor Microenvironment of Primary Central Nervous System Lymphomas and Their Clinical Relevance.

Applied immunohistochemistry &amp; molecular morphology : AIMM
2025

Plasmablast-like lymphoma cells as a distinct subpopulation confer multidrug resistance in PCNSL.

Neuro-oncology
2025

Primary central nervous system lymphoma: Predictors of response to induction therapy and patterns of progression.

Neuro-oncology advances
2025

Refinement of primary central nervous system lymphoma prognostication and response assessment using 3-dimensional MRI.

Neuro-oncology advances
2026

Diffusion-weighted imaging and susceptibility-weighted imaging in differentiating ring-enhancing primary central nervous system lymphoma from infections.

The neuroradiology journal
2025

Primary central nervous system lymphoma: A 7 years single-center experience.

Neuro-Chirurgie
2025

A Case of Primary Central Nervous System T-cell Lymphoma of the Spinal Cord Detected by 18 F-FDG PET/CT.

Clinical nuclear medicine
2025

Comparison of survival and progression after focal- or whole brain radiotherapy in patients with primary CNS lymphoma - Results from a large multicenter analysis of the German Society of Radiation Oncology's Neuro-Radio-Oncology Working Group (DEGRO AG-NRO).

Radiotherapy and oncology : journal of the European Society for Therapeutic Radiology and Oncology
2025

Large B-cell Lymphoma With Interferon Regulatory Factor 4 Rearrangement Presenting as a Primary Central Nervous System Lymphoma in the Brain.

Cureus
2025

Identifying risk factors for methotrexate-induced acute kidney injury despite full prevention in patients with primary central nervous system lymphoma.

Toxicology and applied pharmacology
2025

Clinical outcomes of newly diagnosed PCNSL treated with rituximab-methotrexate-cytarabine with or without ibrutinib: a retrospective study.

Frontiers in immunology
2025

Radiomics-Based Differentiation of Primary Central Nervous System Lymphoma and Solitary Brain Metastasis Using Contrast-Enhanced T1-Weighted Imaging: A Retrospective Machine Learning Study.

Academic radiology
2025

Population pharmacokinetics of high-dose methotrexate in patients with primary central nervous system lymphoma.

Frontiers in pharmacology
2025

Single-cell and spatial characterization of plasmablast-like lymphoma cells in primary central nervous system lymphoma.

Blood neoplasia
2025

Novel Therapies in Primary Central Nervous System Lymphoma.

Clinical pharmacology : advances and applications
2025

The activation of the NF-κB p65/PD-L1 signaling pathway in AIDS-related PCNSL is related to TNF-α and IFN-γ but not to MYD88 and CD79B mutations.

Pathology, research and practice
2025

Primary Diffuse Large B-Cell Lymphoma of the Central Nervous System-Outcomes in Finland: A Nationwide Population-Based Study.

EJHaem
2025

Liquid Biopsy in B and T Cell Lymphomas: From Bench to Bedside.

International journal of molecular sciences
2025

Orelabrutinib combined with rituximab and high-dose methotrexate as induction therapy in newly diagnosed primary central nervous system lymphoma.

Investigational new drugs
2025

Post-marketing surveillance of tirabrutinib in 189 patients with r/r primary central nervous system lymphoma.

Future oncology (London, England)
2025

OCT4 and Nestin Expression in the Microenvironment of Primary Central Nervous System Lymphomas.

International journal of hematology-oncology and stem cell research
2025

Complex Differential Diagnosis Between Cerebral Toxoplasmosis and Primary CNS Lymphoma in Immunocompromised Patients: The Value of Brain Biopsy.

Turkish neurosurgery
2025

Diagnostic Challenges in an Elderly Patient With Cognitive Decline and an Incidental Brain Mass.

Cureus
2025

Accumulation of N-Isopropyl-p(123I)iodoamphetamine on Single-Photon Emission Computed Tomography Between Immunosuppressed and Non-immunosuppressed Patients With Primary Central Nervous System Lymphoma.

Cureus
2025

Efficacy and safety of the R2-MTX regimen in primary central nervous system lymphoma (PCNSL): a single-center retrospective analysis.

Journal of cancer research and clinical oncology
2025

Establishment and evaluation of an automatic multi?sequence MRI segmentation model of primary central nervous system lymphoma based on the nnU?Net deep learning network method.

Oncology letters
2025

Metabolic pathway alterations in cerebrospinal fluid as diagnostic biomarkers for primary central nervous system lymphoma.

Clinica chimica acta; international journal of clinical chemistry
2025

Mild Metabolism Increase on Posttreatment 18 F-FDG PET may Indicate Recurrence of Primary CNS Lymphoma.

Clinical nuclear medicine
2025

The "purfling sign": a new imaging marker for the diagnosis of primary CNS lymphoma.

La Radiologia medica
2025

Role of Radiotherapy in PCNSL within the Current Therapeutic Landscape: a Comprehensive Review.

Current treatment options in oncology
2025

Development and validation of a preoperative magnetic resonance imaging-based and machine learning model for the noninvasive differentiation of intracranial glioblastoma, primary central nervous system lymphoma and brain metastases: a retrospective analysis.

Frontiers in oncology
2025

Primary vitreoretinal lymphoma: diagnosis, treatment, and prognosis-a review of current knowledge and future directions.

Blood science (Baltimore, Md.)
2025

Prediction of early recurrence in primary central nervous system lymphoma based on multimodal MRI-based radiomics: A preliminary study.

European journal of radiology
2025

A Natural Course From Primary Intraocular Lymphoma to Brain Lymphoma in Four Years According to Patient's Choice.

Cureus
2025

Prospective phase II trial of first-line rituximab, methotrexate, and orelabrutinib (R-MO) in primary central nervous system lymphoma.

Blood cancer journal
2025

Safety and efficacy of programmed cell death-1 inhibitors in relapsed immune-privileged site lymphoma: A systematic review and meta-analysis.

PloS one
2025

Whole brain radiotherapy combined with CART-cell therapy for relapsed/refractory central nervous system B-cell lymphoma.

Annals of hematology
2025

PFS24 as a prognostic milestone in patients with newly diagnosed primary CNS lymphoma.

Journal of hematology &amp; oncology
2025

Machine Learning-Enhanced Cerebrospinal Fluid N-Glycome for the Diagnosis and Prognosis of Primary Central Nervous System Lymphoma.

Journal of proteome research
Ver todos os 1.986 no EuropePMC

Associações

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Doença com base genética

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Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ainda não achamos doenças com sintomas parecidos o suficiente.

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

É um tipo de linfoma não-Hodgkin ou de Hodgkin que se forma primariamente no cérebro ou na medula espinhal. No instante do diagnóstico, não existe disseminação ou presença da lesão tumoral fora do sistema nervoso central.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

4 fontes citadas no texto · 10 publicações do grafo RarasNet (PubMed) · 5 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. MONDO
    purl.obolibrary.org
  2. Primary central nervous system lymphoma: Evolving treatment strategies to achieve improved long-term disease control.
    Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners2026PMID 41841431
  3. GARD:9318
    GARD (NIH)
  4. Q3246818
    Wikidata

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Citar este verbete

Raras. (2026). Linfoma primário do sistema nervoso central. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/linfoma-primario-do-sistema-nervoso-central

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Linfoma primário do sistema nervoso central
Compêndio · Raras BR

Linfoma primário do sistema nervoso central

ORPHA:46135 · MONDO:0002571
Prevalência
1-9 / 1 000 000
Herança
Not applicable
CID-10
C83.3 · Linfoma não-Hodgkin difuso, grandes células (difuso)
CID-11
Ensaios
57 ativos
Início
Adult, Elderly
Prevalência
0.44 (United States)
MedGen
UMLS
C0280803
EuropePMC
Wikidata
Wikipedia
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Dado público estruturado
fonte: Wikidata
Medicamentos aprovados FDA
fonte: FDA OpenFDA
Rara