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Primary breast diffuse large B‐cell lymphoma ( PB ‐ DLBCL ) is a rare subtype of  DLBCL  with limited data on patterns of failure. This multicenter study aimed to define the optimum treatment strategy and patterns of failure for  PB ‐ DLBCL  patients. We retrospectively reviewed data on 108  PB ‐ DLBCL  patients from 21 Chinese medical centers. Only patients with localized disease (involvement of breast and localized lymph nodes) were included. After a median follow‐up of 3.2 years, 32% of patients developed progression or relapse. A continuous pattern of relapse was observed, characterized by frequent late relapses in the contralateral breast and central nervous system ( CNS ). Although rituximab significantly reduced the overall cumulative risk of progression or relapse (5‐year cumulative risk 57% vs 24%,  P  =   .029), it had limited effect on the reduction of breast relapse ( P  =   .46). Consolidative radiotherapy significantly decreased the risk of breast relapse, even in the subgroup of patients treated with rituximab (5‐year cumulative risk 21.2% vs 0%,  P  =   .012). A continuous risk of  CNS  progression or relapse up to 8.2 years from diagnosis was observed (10‐year cumulative risk 28.3%), with a median time to  CNS  relapse of 3.1 years. Neither rituximab nor prophylactic intrathecal chemotherapy significantly decreased the risk of  CNS  relapse. In summary, our study indicates that  PB ‐ DLBCL  has a continuous pattern of relapse, especially with frequent late relapses in the  CNS  and contralateral breast. Rituximab and  RT  confer complementary benefit in the reduction of relapse. However, neither the addition of rituximab nor prophylactic intrathecal chemotherapy could effectively prevent  CNS  relapse for  PB ‐ DLBCL  patients.

Primary breast diffuse large B‐cell lymphoma in the rituximab era: Therapeutic strategies and patterns of failure