# Linfoma primário do sistema nervoso central

> Página oficial: https://raras.org/doenca/linfoma-primario-do-sistema-nervoso-central
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 46135 — https://www.orpha.net/en/disease/detail/46135
- **CID-10**: C83.3

## Descrição clínica

A non-Hodgkin or Hodgkin lymphoma that arises in the brain or spinal cord as a primary lesion. There is no evidence of lymphoma outside the central nervous system at the time of diagnosis.

## Epidemiologia e herança

- **Prevalência**: 1-9 / 1 000 000
- **Padrão de herança**: Not applicable

## Ensaios clínicos ativos (34)

- **NCT07137494** [RECRUITING]: A Study of MB-CART19.1 Cellular Therapy for People With Central Nervous System Lymphoma (CNSL) — https://clinicaltrials.gov/study/NCT07137494
- **NCT04609046** [RECRUITING]: Testing the Addition of Lenalidomide and Nivolumab to the Usual Treatment for Primary CNS Lymphoma — https://clinicaltrials.gov/study/NCT04609046
- **NCT03328078** [RECRUITING]: CA-4948-101: Open-Label, Dose Escalation and Expansion Trial of Emavusertib (CA-4948) in Relapsed or Refractory Primary Central Nervous System Lymphoma (R/R PCNSL) — https://clinicaltrials.gov/study/NCT03328078
- **NCT07523737** [RECRUITING]: Pomalidomide, Anti-PD-1 Antibody Combined With Selinexor (PPS) in Relapsed/Refractory Primary Central Nervous System Diffuse Large B-Cell Lymphoma — https://clinicaltrials.gov/study/NCT07523737
- **NCT05131022** [RECRUITING]: A Study of NX-5948 in Adults With Relapsed/Refractory B-cell Malignancies — https://clinicaltrials.gov/study/NCT05131022
- **NCT07015242** [RECRUITING]: A Study of the Efficacy and Safety of Lisocabtagene Maraleucel (Liso-cel) as First-Line Therapy in Adults With Transplant-Ineligible Primary Central Nervous System Lymphoma — https://clinicaltrials.gov/study/NCT07015242
- **NCT04830137** [RECRUITING]: A Study of NX-2127 in Adults With Relapsed/Refractory B-cell Malignancies — https://clinicaltrials.gov/study/NCT04830137
- **NCT07104032** [RECRUITING]: IGNITE: Study of Tirabrutinib vs Rituximab/Temozolomide for Relapsed/Refractory Primary Central Nervous System Lymphoma (PCNSL) — https://clinicaltrials.gov/study/NCT07104032
- **NCT05256641** [RECRUITING]: Acalabrutinib Maintenance for the Treatment of Patients With Large B-cell Lymphoma — https://clinicaltrials.gov/study/NCT05256641
- **NCT07350850** [RECRUITING]: A Multicenter Two-Cohort Study of Methotrexate, Rituximab, Sintilimab and Pirtobrutinib for Treatment-Naive PCNSL vs. Real-World Investigator-Selected Treatment (Observational Cohort) — https://clinicaltrials.gov/study/NCT07350850

## Importante

O Raras **não diagnostica e não prescreve**. Esta página é educativa e informativa.
Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

---

**Citação sugerida**: Raras.org — Linfoma primário do sistema nervoso central. Disponível em: https://raras.org/doenca/linfoma-primario-do-sistema-nervoso-central
**Formato HTML**: https://raras.org/doenca/linfoma-primario-do-sistema-nervoso-central
**Formato RDF/Turtle**: https://raras.org/api/rdf?orpha=46135