Understand this diagnosis through data, infographics, and evidence based on modern classification and current research.
Memory loss, personality changes, confusion, executive dysfunction. Most common presentation due to deep white matter and periventricular location.
Hemiparesis, aphasia, visual field deficits depending on tumor location. Often multifocal or deep-seated lesions.
Headache, nausea, vomiting, papilledema from mass effect or hydrocephalus.
Blurred vision, floaters, decreased visual acuity. ~15-25% of PCNSL involves the eyes (vitreoretinal lymphoma).
Less common than in other brain tumors but can occur, especially with cortical involvement.
Cranial nerve palsies, radiculopathy from leptomeningeal involvement. CSF cytology may show lymphoma cells.
Contrast-enhanced MRI reveals homogeneously enhancing deep periventricular lesion(s). Often multifocal. DWI shows restricted diffusion.
Tissue diagnosis via stereotactic biopsy (NOT resection). Corticosteroids should be avoided before biopsy as they cause tumor lysis.
Slit-lamp examination and vitreous biopsy to evaluate for vitreoretinal lymphoma involvement (~15-25% of cases).
Lumbar puncture for cytology, flow cytometry, protein, and MYD88 mutation testing. Positive in ~15-30%.
CT chest/abdomen/pelvis, bone marrow biopsy, and testicular ultrasound (men >60) to exclude systemic lymphoma.
MYD88 L265P and CD79B mutation testing, cell-of-origin (Hans algorithm), MYC/BCL2/BCL6 FISH.
Backbone of PCNSL treatment. HD-MTX (3.5-8 g/m²) crosses the blood-brain barrier. Often combined with rituximab, cytarabine, and/or thiotepa (MATRix, R-MPV regimens).
Anti-CD20 monoclonal antibody added to HD-MTX backbone. R-MPV (rituximab, MTX, procarbazine, vincristine) and MATRix are standard induction regimens.
High-dose chemotherapy with thiotepa-based conditioning followed by autologous stem cell transplant (ASCT). Increasingly preferred over WBRT for consolidation.
Ibrutinib, zanubrutinib, and pirtobrutinib show high response rates in relapsed PCNSL, especially with MYD88/CD79B mutations. Oral agents with CNS penetration.
Historically used for consolidation but associated with neurocognitive toxicity. Now typically reserved for elderly or transplant-ineligible patients at reduced doses.
Novel agents: CAR-T therapy, bispecific antibodies, lenalidomide, checkpoint inhibitors, and novel combinations for relapsed/refractory disease.
Find matching trials →Primary specialist for surgical resection and skull base approaches
Plans and delivers radiation therapy and stereotactic radiosurgery
For higher-grade or refractory cns lymphomas requiring systemic therapy
Specialized imaging interpretation and monitoring
Tissue analysis, WHO grading, and molecular profiling
Guides you through appointments, insurance, and logistics
Education, support, and research funding for all lymphoma types including PCNSL.
Visit Lymphoma Research Foundation →Leading nonprofit investing in brain tumor research, advocacy, and patient support.
Visit National Brain Tumor Society →Patient support, financial assistance, and clinical trial navigation for blood cancer patients.
Visit Leukemia & Lymphoma Society →Evidence-based clinical practice guidelines for CNS lymphoma management.
Visit NCCN Guidelines (CNS Cancers) →