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7 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis and staging | 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. | not mapped |
| First-line treatment of stage I and II disease | 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. | not mapped |
| What happens at relapse | 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. | not mapped |