The first 60 days: Primary CNS lymphoma
Primary CNS lymphoma is a lymphoma confined to the brain, eyes and spinal fluid. Unlike most brain tumours it is chemo-sensitive: high-dose methotrexate-based treatment cures a substantial minority, and consolidation with a stem-cell transplant has replaced whole-brain radiation for the fit. Below, week by week, is what OnCo's record of Primary CNS lymphoma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Rituximab + high-dose methotrexate + cytarabine ± thiotepa (MATRix) ×4, then high-dose thiotepa-based chemotherapy with autologous transplant (IELSG32, IELSG43).
- Newly diagnosed, older/unfitEANO/ESMO PCNSL guideline
High-dose methotrexate with temozolomide, procarbazine or rituximab (e.g. MT-R, R-MP), then maintenance (temozolomide, lenalidomide) or reduced-dose WBRT; ibrutinib-based induction in trials.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- Medical oncologistNamed in the standard of care for: Newly diagnosed, fit (<65-70), Newly diagnosed, older/unfit, Relapsed/refractory.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Newly diagnosed, older/unfit.
- Transplant and cell therapy teamNamed in the standard of care for: Newly diagnosed, fit (<65-70), Relapsed/refractory.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
Re-induction with methotrexate if durable first remission; ibrutinib, lenalidomide-rituximab, high-dose chemotherapy/ASCT if not done, WBRT; CD19 CAR-T and PD-1 inhibitors in trials or off-label.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example MYD88 L265P and CD79B mutations, CSF cytology and flow cytometry, IL-10 in CSF/vitreous, MSKCC and IELSG prognostic scores, Slit-lamp examination for ocular involvement), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Immunocompetent PCNSL, AIDS-related / post-transplant PCNSL, Primary vitreoretinal lymphoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Newly diagnosed, fit (<65-70)
- For my situation (newly diagnosed, fit (<65-70)), which of the standard options do you recommend and why?Guideline options include: Rituximab + high-dose methotrexate + cytarabine ± thiotepa (MATRix) ×4, then high-dose thiotepa-based chemotherapy with autologous transplant (IELSG32, IELSG43).
- Am I a candidate for Methotrexate, Rituximab, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Newly diagnosed, older/unfit
- For my situation (newly diagnosed, older/unfit), which of the standard options do you recommend and why?Guideline options include: High-dose methotrexate with temozolomide, procarbazine or rituximab (e.g. MT-R, R-MP), then maintenance (temozolomide, lenalidomide) or reduced-dose WBRT; ibrutinib-based induction in trials.
- Am I a candidate for Methotrexate, Temozolomide, Rituximab or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Relapsed/refractory
- For my situation (relapsed/refractory), which of the standard options do you recommend and why?Guideline options include: Re-induction with methotrexate if durable first remission; ibrutinib, lenalidomide-rituximab, high-dose chemotherapy/ASCT if not done, WBRT; CD19 CAR-T and PD-1 inhibitors in trials or off-label.
- Am I a candidate for Ibrutinib, Lenalidomide, Axicabtagene ciloleucel or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Ibrutinib, Axicabtagene ciloleucel, Lenalidomide, Zamtocabtagene autoleucel?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Most patients are over 65 and cannot tolerate curative-intent therapy”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Neurocognitive decline from disease and therapy”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Primary CNS lymphoma: the full pagePrimary CNS lymphoma is a lymphoma confined to the brain, eyes and spinal fluid. Unlike most brain tumours it is chemo-sensitive: high-dose methotrexate-based treatment cures a substantial minority, and consolidation with a stem-cell transplant has replaced whole-brain radiation for the fit.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Cell of origin (GCB vs ABC): Whether a large B-cell lymphoma resembles a germinal-centre B cell or an activated B cell; the activated type does worse and depends on different pathways.
- Blood-brain barrier (BBB): The tight seal around brain blood vessels that keeps most drugs out, one of the two main reasons brain cancer is so hard to treat.
Every term links to the glossary.