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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.
Primary mediastinal large B-cell lymphoma is a distinct entity in the WHO classification, arising from thymic medullary B cells and sharing biology with nodular sclerosis classical Hodgkin lymphoma: gains and rearrangements of 9p24.1 (PD-L1, PD-L2, JAK2), CIITA rearrangements with loss of MHC class II, JAK-STAT and NF-kappa-B activation, and weak CD30 expression. It expresses CD20, CD23 and MAL, typically lacks surface immunoglobulin, and presents as a bulky anterior mediastinal mass in patients with a median age around 35, with local spread to lung, pleura and pericardium but rarely to marrow. Mediastinal grey zone lymphoma sits between it and Hodgkin lymphoma.
First-line treatment is rituximab-based immunochemotherapy. The National Cancer Institute series of dose-adjusted EPOCH-R (Dunleavy, NEJM 2013) treated 51 patients without radiotherapy with event-free survival of 93 percent and overall survival of 97 percent, and DA-EPOCH-R became the regimen that lets young patients avoid mediastinal radiotherapy; R-CHOP with consolidation radiotherapy is the alternative. The IELSG37 trial (2024) randomised patients in complete metabolic response on end-of-treatment PET to radiotherapy or observation and showed no loss of disease control without radiotherapy (30-month progression-free survival 96.7 versus 98.5 percent), so PET now decides who is irradiated and most patients are spared the heart and breast cancer risks of chest radiotherapy.
Relapsed or refractory disease, about 10 to 15 percent of patients, is treated with salvage chemotherapy and autologous transplant when chemosensitive, and the 9p24.1 lesion makes it one of the most immunotherapy-sensitive B-cell lymphomas: pembrolizumab produced a 45 percent response rate in KEYNOTE-170 and was approved in June 2018, the first drug licensed specifically for this lymphoma; nivolumab plus brentuximab vedotin gave a 73 percent response rate in CheckMate 436; and the CD19 CAR-T products axicabtagene ciloleucel and lisocabtagene maraleucel are licensed for large B-cell lymphoma including primary mediastinal disease. Open questions are how to identify the few patients who fail first-line therapy early, whether checkpoint blockade belongs in first line, and how to reduce the late effects of anthracycline and radiotherapy in survivors who are mostly in their thirties.
About 2 to 4 percent of non-Hodgkin lymphomas, arising from thymic B cells in young adults, mostly women in their thirties, who present with a bulky anterior chest mass, cough, superior vena cava obstruction or breathlessness; most are cured with first-line immunochemotherapy.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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.
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.
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End-of-treatment PET now decides radiotherapy in primary mediastinal B-cell lymphoma: a negative scan means no radiotherapy, whichever chemotherapy regimen was used.
Every lymphoma diagnosis on this site refers to an entity in this classification or the parallel International Consensus Classification.
The piece that turns a research classification into something a trial can use on one person's biopsy, with a probability attached rather than a flat label. It is the reason genetics-directed lymphoma trials became possible at all.
The evidence that made lisocabtagene maraleucel the CD19 CAR-T product most often chosen for older or frailer patients, because its severe cytokine release syndrome rate is a fraction of the other two products'.
Pembrolizumab is approved for relapsed primary mediastinal B-cell lymphoma and is the usual bridge to CAR-T or transplant in chemotherapy-refractory patients.
Dose-adjusted EPOCH-R without radiotherapy became a standard for primary mediastinal B-cell lymphoma, particularly in the United States, and the IELSG37 trial later confirmed radiotherapy can be omitted after a negative PET.
Query for this cancer: (TITLE:"Primary mediastinal thymic large B-cell lymphoma" OR ABSTRACT:"Primary mediastinal thymic large B-cell lymphoma" OR TITLE:"PMBCL" OR ABSTRACT:"PMBCL" OR TITLE:"PMBL" OR ABSTRACT:"PMBL" OR TITLE:"Primary mediastinal large B-cell lymphoma" OR ABSTRACT:"Primary mediastinal large B-cell lymphoma" OR TITLE:"Thymic large B-cell lymphoma" OR ABSTRACT:"Thymic large B-cell lymphoma" OR TITLE:"Mediastinal grey zone lymphoma related" OR ABSTRACT:"Mediastinal grey zone lymphoma related") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Primary mediastinal (thymic) large B-cell lymphoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Lymphoma Action says a small number of people have more serious problems such as seizures or swelling of the brain, treated with steroids and intensive care, and that most improve within a few days of treatment starting. This is 999.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Breathing very fast, confusion or slurred speech, blue, pale or blotchy skin, a very high or very low temperature, shivering, or a rash that does not fade when pressed: the NHS says call 999 or go to A and E, and do not drive yourself.
A rising potassium level can disturb the heart rhythm, which is the reason blood is checked frequently during the first cycle. The triage standard sends chest pain or tightness straight to 999 whatever the cause.
Fever of 38 C or higher is grade 1 CRS; fever with low blood pressure, fast heartbeat, breathlessness or low oxygen is grade 2 or higher. The labels carry a boxed warning and say to report fever immediately.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
See all on the product pages:Axicabtagene ciloleucelCD20 bispecific antibodies: step-up dosing, fixed duration and what it is like to take oneCentral venous access (port, PICC line)CyclophosphamideCytokine release syndrome (CRS)Cytokine release syndrome and ICANS: grading and managementDoxorubicinEtoposideFebrile neutropeniaHypogammaglobulinaemia and infection risk after B-cell therapiesICANS (neurotoxicity)Lisocabtagene maraleucelNeutropeniaNivolumabPembrolizumabThe CAR-T pathway in lymphoma: referral, apheresis, bridging and the waitingTumour lysis syndrome (TLS)Tumour lysis syndrome in lymphoma: who is at risk, and rasburicaseVincristine·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Primary mediastinal (thymic) large B-cell lymphoma, then print the one-page appointment sheet with room for the answers.
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