Primary mediastinal (thymic) large B-cell lymphoma: lines of therapy

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5 standard-of-care settings across 4 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

Line	All comers	FRα
Advanced, first line	2	·
Second line	1	·
Third line and beyond	·	1
Other settings	1	·

Advanced, first line

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	First line	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).	RituximabDoxorubicinCyclophosphamideEtoposideVincristinePrednisoneR-CHOP (lymphoma chemoimmunotherapy)FDG PETPET-adapted (response-adapted) therapyIELSG37	NCCN Guidelines: B-Cell Lymphomas	88
All comers	Primary mediastinal B-cell lymphoma: which first-line regimen, and why radiotherapy is now usually avoided	A disease of young adults, more often women, presenting with a bulky mass between the lungs that can compress the superior vena cava. It is biologically closer to Hodgkin lymphoma than to diffuse large B-cell lymphoma, which is why PD-1 blockade works in it. Dose-adjusted EPOCH-R for six cycles is the regimen most used in the United States, on the strength of a National Cancer Institute series in which five-year event-free survival was 93 per cent and overall survival 97 per cent, with radiotherapy avoided in 96 per cent of patients. R-CHOP with consolidation radiotherapy is the alternative used in much of Europe. There has never been a randomised comparison of the two. What has been settled is the radiotherapy. IELSG37 randomised patients with a negative end-of-treatment PET to mediastinal radiotherapy or observation: 30-month progression-free survival was 96.2 per cent with observation against 98.5 per cent with radiotherapy, non-inferior, and overall survival was 99 per cent in both arms. Since the patients are young and the field sits over the heart and breasts, avoiding it matters for the next forty years. Radiotherapy is still given where the end-of-treatment PET is positive.	Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphomaIELSG37: omission of radiotherapy in primary mediastinal B-cell lymphoma after a negative PET scanR-CHOP (lymphoma chemoimmunotherapy)RituximabEtoposideDoxorubicinCyclophosphamideVincristinePrednisoneFDG PETDeauville score and PET-adapted therapyRadiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapyLate effects of Hodgkin lymphoma treatment, and the follow-up that answers themFertility before lymphoma treatment: what to ask for, and when	NCCN B-Cell Lymphomas; ESMO; IELSG37	84

Second line

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Primary mediastinal B-cell lymphoma that relapses or does not respond	Relapse is uncommon and almost always early, within the first year. CD19 CAR-T is the treatment of choice and primary mediastinal disease was included in the pivotal ZUMA-1 and TRANSCEND populations. Where CAR-T is not available or has failed, pembrolizumab has an established role: KEYNOTE-170 treated 53 patients whose disease had relapsed after autologous transplant or who could not have one after two or more lines, and reported an objective response of 45 per cent and complete response of 13 per cent, which led to accelerated approval in the United States on 13 June 2018, converted to traditional approval on 14 October 2020. Nivolumab with brentuximab vedotin is the other checkpoint-based option. Salvage chemotherapy with autologous transplant is used where the disease is still chemosensitive and CAR-T is not accessible. Mediastinal radiotherapy is added to a residual localised site.	KEYNOTE-170PembrolizumabNivolumabBrentuximab vedotinAxicabtagene ciloleucelLisocabtagene maraleucelCAR-T cell therapyAutologous stem cell transplant (high-dose therapy)The CAR-T pathway in lymphoma: referral, apheresis, bridging and the waitingRadiotherapy in lymphoma: involved-site fields, 24 Gy, 4 Gy and total skin electron therapy	NCCN B-Cell Lymphomas; KEYNOTE-170	98

Third line and beyond

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
FRα	Relapsed or refractory	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.	Autologous stem cell transplant (high-dose therapy)PembrolizumabKEYNOTE-170NivolumabBrentuximab vedotinAxicabtagene ciloleucelLisocabtagene maraleucelCAR-T cell therapy	NCCN Guidelines: B-Cell Lymphomas	98

Other settings

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Residual PET-positive disease after immunochemotherapy	Biopsy where feasible; involved-site radiotherapy to the mediastinum (30 to 36 Gy) for persistent uptake; salvage therapy for proven refractory disease.	IMRT / IGRT (modern external beam)Proton therapyFDG PETDeauville five-point scale	NCCN Guidelines: B-Cell Lymphomas	93

Regimens in the library

DA-EPOCH-R

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.