Primary CNS Lymphoma
Primary CNS Lymphoma: Symptoms, Causes, Diagnosis, Treatment, and Future Outlook.
Disclaimer:
This blog is for informational purposes only and should not be taken as medical advice. Content is sourced from third parties, and we do not guarantee accuracy or accept any liability for its use. Always consult a qualified healthcare professional for medical guidance.
What is Primary CNS Lymphoma?
Primary Central Nervous System Lymphoma (PCNSL) is a rare, aggressive non-Hodgkin lymphoma confined to the brain, spinal cord, eyes, or meninges, without systemic involvement. It’s mostly B-cell (95%), with T-cell rare. In 2025, ~1,500 US cases, median age 65, more in immunocompromised (HIV, transplants).
Symptoms
Symptoms include headaches, nausea/vomiting, seizures, confusion, personality changes, weakness/paralysis, vision problems, speech difficulties, and balance issues. Ocular involvement causes blurred vision/floaters. Symptoms mimic stroke or dementia.
Causes
Causes involve immune dysregulation, with EBV in immunocompromised. Risk factors include HIV (300-fold increase), immunosuppression, and age. In 2025, genetic mutations (MYD88, CD79B) are key in immunocompetent cases.
Diagnosis
Diagnosis uses MRI showing enhancing lesions, CSF analysis for lymphoma cells, stereotactic biopsy for confirmation, and ophthalmic exam for eye involvement. In 2025, liquid biopsies improve non-invasive diagnosis.
Treatment
Treatment uses high-dose methotrexate-based chemotherapy, often with rituximab. Whole-brain radiation for consolidation, but targeted (ibrutinib for MYD88+) and immunotherapy (nivolumab) emerge. SCT for young patients. In 2025, CAR-T shows 50% response.
Future Outlook
In 2025, median survival is 3-5 years with chemo, better in young (60%). Targeted therapies extend to 5 years. By 2030, CAR-T and inhibitors could achieve 70% survival.
Sources
The information is based on Barrow Neurological’s “Central Nervous System Lymphoma – Symptoms and Treatment” for symptoms; NCI’s “Primary CNS Lymphoma Treatment (PDQ®)” for treatment; Brain Tumour Research’s “Primary CNS lymphoma | Brain Tumour Types” for types; PMC’s “Central Nervous System Lymphoma” for overview; NORD’s “Primary Central Nervous System Lymphoma” for symptoms; UCI Health’s “Primary Central Nervous System (CNS) Lymphoma” for symptoms; Macmillan’s “Primary CNS lymphoma (PCNSL)” for symptoms; PMC’s “Primary lymphoma of the central nervous system: epidemiology” for epidemiology; Lymphoma Action’s “Central nervous system (CNS) lymphoma” for overview.