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A large B-cell lymphoma that starts in a testicle rather than in a lymph node, usually in a man over 60, and shows itself as a painless swelling. It is the commonest cancer of the testicle in older men, and it behaves as one disease with lymphoma of the brain and of the eye, which is why treatment deliberately protects both.
What it is. A diffuse large B-cell lymphoma arising inside a testicle. It presents as a firm, usually painless swelling, and it is the commonest malignant tumour of the testis in men over 60, an age at which the germ cell tumours of younger men have become rare. The diagnosis is usually made on the testicle after it has been removed, because a solid testicular mass is removed rather than biopsied.
How it differs from its family, and this is the whole of the management. The testis is an immune-privileged site: a barrier of cells keeps the immune system out, which protects developing sperm from being attacked and also shelters a lymphoma from immune surveillance and from many drugs. The brain and the inside of the eye are protected in the same way. WHO-HAEM5 recognised in 2022 that large B-cell lymphomas at those three sites are one entity, which it called primary large B-cell lymphoma of immune-privileged sites, because they share an activated B-cell phenotype, concurrent MYD88 and CD79B mutations, loss of the machinery that displays antigen to the immune system, and a habit of relapsing in each other: a testicular lymphoma comes back in the brain or in the other testicle, and a lymphoma of the eye follows or precedes one in the brain.
Where the two classifications disagree. The International Consensus Classification discussed the same grouping and decided it was premature, on the grounds that lymphomas at some of those sites are heterogeneous and that a pathologist often does not know whether other sites are involved. It nevertheless recognises primary diffuse large B-cell lymphoma of the testis as a specific entity in its own right, closely related to lymphoma of the central nervous system and sharing the same genetic subgroup. So the two books agree about the biology and differ about the filing.
How it is treated, and why three things are done at once. The trial that set the standard, IELSG-10, treated 53 men with stage I or II disease with six to eight cycles of rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone, four doses of methotrexate into the spinal fluid, and 30 Gy of radiotherapy to the remaining testicle, with the regional lymph nodes irradiated in stage II. At a median follow-up of 65 months, five-year progression-free survival was 74 per cent and overall survival 85 per cent. Ten patients relapsed, three of them in the central nervous system, giving a five-year cumulative incidence of relapse in the brain or spinal cord of 6 per cent. No patient relapsed in the remaining testicle. Those last two numbers are the reason the regimen looks the way it does.
What is still unsettled. Irradiating the remaining testicle prevents relapse there but causes infertility and low testosterone, which matters to a minority of men who are diagnosed young. Whether methotrexate into the spinal fluid is the right prophylaxis against disease in the brain, or whether high-dose intravenous methotrexate would do better, has not been settled by a randomised trial. In the SEER analysis covering 1980 to 2005, survival in testicular lymphoma did not improve after rituximab came into use in the way it did in lymphoma of the lymph nodes, which the authors noted explicitly.
In the United States SEER registries, 769 men with primary testicular diffuse large B-cell lymphoma were identified between 1980 and 2005, with a median age at diagnosis of 68.0 years and an incidence that rose over the period; a later SEER analysis identified 1,169 patients between 1973 and 2013, median age 70 years, of whom 82.9 per cent had diffuse large B-cell lymphoma and 68.6 per cent had stage I or II disease at diagnosis. The corpus does not quote a United Kingdom incidence figure, because no source it could verify publishes one for this entity.
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, 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), Primary mediastinal (thymic) large B-cell lymphoma, 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
No subtypes recorded beyond the ones named in the family strip above.
A solid testicular mass is removed through the groin rather than biopsied, so the diagnosis is usually made on the removed testicle. Staging then has to cover the sites this disease travels to: computed tomography or PET-CT of the body, imaging of the brain, examination of the spinal fluid, and examination of the remaining testicle. Sperm banking is discussed before treatment where it is relevant, because radiotherapy to the remaining testicle causes infertility and low testosterone.
Rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone for six to eight cycles, methotrexate into the spinal fluid, and radiotherapy to the remaining testicle. In IELSG-10, which treated 53 men this way, five-year progression-free survival was 74 per cent and overall survival 85 per cent at a median follow-up of 65 months; the five-year cumulative incidence of relapse in the central nervous system was 6 per cent and there were no relapses in the irradiated testicle. Grade 3 or 4 neutropenia occurred in 28 per cent and infection in 4 per cent.
Relapse is most often in the central nervous system, and it is treated on the pathway for lymphoma of the brain rather than on the pathway for nodal lymphoma: regimens built around high-dose methotrexate that crosses into the brain, and consideration of high-dose therapy with an autologous stem cell transplant using a conditioning regimen that also reaches the brain. The detail is on the primary central nervous system lymphoma page.
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Query for this cancer: (TITLE:"Primary large B-cell lymphoma of the testis" OR ABSTRACT:"Primary large B-cell lymphoma of the testis" OR TITLE:"Primary testicular lymphoma" OR ABSTRACT:"Primary testicular lymphoma" OR TITLE:"PTL" OR ABSTRACT:"PTL" OR TITLE:"Primary testicular diffuse large B-cell lymphoma" OR ABSTRACT:"Primary testicular diffuse large B-cell lymphoma" OR TITLE:"Testicular lymphoma" OR ABSTRACT:"Testicular lymphoma" OR TITLE:"Primary diffuse large B-cell lymphoma of the testis" OR ABSTRACT:"Primary diffuse large B-cell lymphoma of the testis") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Primary large B-cell lymphoma of the testis, not a curated reading list.
Across 769 men in the SEER registries diagnosed between 1980 and 2005, median overall survival was 4.6 years and disease-specific survival was 71.5 per cent at three years, 62.4 per cent at five and 43.0 per cent at fifteen. Unlike nodal diffuse large B-cell lymphoma, disease-specific survival did not improve after the year 2000, when rituximab came into use.
Fifty-three men with stage I or II disease treated with rituximab-containing chemotherapy, methotrexate into the spinal fluid and radiotherapy to the remaining testicle: five-year progression-free survival 74 per cent, overall survival 85 per cent, no relapse in the irradiated testicle and a 6 per cent five-year risk of relapse in the central nervous system.
WHO-HAEM5 created primary large B-cell lymphoma of immune-privileged sites, covering the central nervous system, the vitreoretina and the testis. The International Consensus Classification judged the grouping premature but recognised testicular lymphoma as an entity closely related to the central nervous system disease.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Fatal if given intrathecally: label all syringes.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
When a large, fast-growing lymphoma breaks up quickly, the contents of the cells flood the blood and can stop the kidneys or the heart. It is predictable, preventable and is the reason the first days of treatment are given in hospital with fluids and blood tests every few hours.
See all on the product pages:CyclophosphamideDoxorubicinMethotrexatePrednisoneVincristine·Printable cards in the navigator
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