What changed: Primary large B-cell lymphoma of the testis

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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.

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.

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.

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.