The first 60 days: Primary mediastinal (thymic) large B-cell lymphoma
Primary mediastinal B-cell lymphoma is a fast-growing lymphoma of the thymus behind the breastbone that mostly affects young women. Immunochemotherapy cures about nine in ten, radiotherapy can now be skipped when the end-of-treatment scan is clear, and PD-1 antibodies and CAR-T cells rescue many of those who relapse. Below, week by week, is what OnCo's record of Primary mediastinal (thymic) large B-cell 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.
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.
- PathologistNamed in the standard of care for: Residual PET-positive disease after immunochemotherapy.
- RadiologistNamed in the standard of care for: First line, Residual PET-positive disease after immunochemotherapy.
- Medical oncologistNamed in the standard of care for: First line, Residual PET-positive disease after immunochemotherapy, Relapsed or refractory.
- Clinical oncologist (radiotherapy)Named in the standard of care for: First line, Residual PET-positive disease after immunochemotherapy.
- Transplant and cell therapy teamNamed in the standard of care for: First line, Relapsed or refractory.
- Palliative and supportive care teamNamed in the standard of care for: First line.
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.
Dose-adjusted EPOCH-R for six cycles without radiotherapy, or R-CHOP for six cycles with PET-guided consolidation radiotherapy; end-of-treatment PET decides whether radiotherapy is needed (IELSG37).
Biopsy where feasible; involved-site radiotherapy to the mediastinum (30 to 36 Gy) for persistent uptake; salvage therapy for proven refractory disease.
Salvage chemotherapy then autologous stem cell transplant if chemosensitive; pembrolizumab (KEYNOTE-170) or nivolumab plus brentuximab vedotin; CD19 CAR-T (axicabtagene ciloleucel, lisocabtagene maraleucel) after two lines or as second line for early relapse.
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 CD20, CD23, MAL and weak or partial CD30 expression, 9p24.1 gain or amplification, CIITA rearrangement and loss of MHC class II, End-of-treatment FDG PETto decide on radiotherapy, Circulating tumour DNA for response monitoring), 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 Classic primary mediastinal B-cell lymphoma, Mediastinal grey zone lymphoma, Relapsed or refractory primary mediastinal B-cell lymphoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
First line
- For my situation (first line), which of the standard options do you recommend and why?Guideline options include: Dose-adjusted EPOCH-R for six cycles without radiotherapy, or R-CHOP for six cycles with PET-guided consolidation radiotherapy; end-of-treatment PET decides whether radiotherapy is needed (IELSG37).
- Am I a candidate for Rituximab, Doxorubicin, Cyclophosphamide or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of IELSG37 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Residual PET-positive disease after immunochemotherapy
- For my situation (residual pet-positive disease after immunochemotherapy), which of the standard options do you recommend and why?Guideline options include: Biopsy where feasible; involved-site radiotherapy to the mediastinum (30 to 36 Gy) for persistent uptake; salvage therapy for proven refractory disease.
Relapsed or refractory
- For my situation (relapsed or refractory), which of the standard options do you recommend and why?Guideline options include: Salvage chemotherapy then autologous stem cell transplant if chemosensitive; pembrolizumab (KEYNOTE-170) or nivolumab plus brentuximab vedotin; CD19 CAR-T (axicabtagene ciloleucel, lisocabtagene maraleucel) after two lines or as second line for early relapse.
- Am I a candidate for Pembrolizumab, Nivolumab, Brentuximab vedotin or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of KEYNOTE-170 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of IELSG37, KEYNOTE-170, ctDNA monitoring in lymphoma (PhasED-seq, clonoSEQ), Glofitamab?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 “No validated way to identify the 10 to 15 percent who will fail first-line therapy before they do”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Checkpoint blockade in first line is untested in randomised trials”. 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 mediastinal (thymic) large B-cell lymphoma: the full pagePrimary mediastinal B-cell lymphoma is a fast-growing lymphoma of the thymus behind the breastbone that mostly affects young women. Immunochemotherapy cures about nine in ten, radiotherapy can now be skipped when the end-of-treatment scan is clear, and PD-1 antibodies and CAR-T cells rescue many of those who relapse.
- 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.
- R-CHOP (lymphoma chemoimmunotherapy): R-CHOP is the standard first treatment for diffuse large B-cell lymphoma: rituximab (an antibody against CD20) plus four chemotherapy drugs (cyclophosphamide, doxorubicin, vincristine, prednisone), given every three weeks for six cycles with curative intent.
- Deauville five-point scale: A 1-to-5 score for how bright a lymphoma looks on PET compared with the liver; 1-3 is considered a complete metabolic response.
- ICANS (neurotoxicity): ICANS is confusion, speech difficulty, and rarely seizures after CAR-T or bispecific therapy.
- Lugano classification / Ann Arbor staging: The Lugano classification is the lymphoma staging system: stage I to IV by how many lymph node regions and organs are involved, with PET-based response criteria.
- Autologous stem cell transplant (ASCT): High-dose chemotherapy (melphalan in myeloma, BEAM in lymphoma) that would permanently destroy the bone marrow, made survivable by giving the patient back their own previously collected stem cells, which engraft in 10-14 days.
- Cytokine release syndrome (CRS): A flood of inflammatory signals when immune cells are activated en masse, causing fever, low blood pressure, and sometimes organ failure.
Every term links to the glossary.