High grade primary breast lymphoma : is it a different clinical entity?

Primary lymphoma of the breast (PBL) is a rare neoplasm, its outcome remains unclear compared to other lymphomas. We performed a retrospective study of 19 cases of high grade PBL. There were 17 Diffuse large B cell lymphoma (DLBCL) and 2 follicular and diffuse grade 3 lymphomas. Four patients were treated with local treatment only, 15 received chemotherapy including 11 treated with CHOP or ACVBP regimens followed by involved field radiotherapy. The actuarial survival for the whole population was 38%. Three of the 4 patients treated only with a local treatment died of their lymphoma. Three patients progressed on therapy and 5 relapsed in the first year of follow-up including 2 central nervous system recurrences. Among the 11 patients treated with chemotherapy, 2 died of their lymphoma. The overall survival of this subgroup was 73% (median follow-up of 57 months). We observed, like others in the literature, a better prognosis for lymphomas co-expressing Bcl6 and CD 10. The treatment should be based on the same modalities, but including a CNS prophylaxis even if poor prognosis factors are lacking. A radical mastectomy increases the risk of treatment failure and has to be avoided.