Primary breast lymphoma (PBL) is a rare disease accounting for 0.4-0.5% of all breast malignancies. The most common histological subtype is diffuse large B-cell lymphoma (DLBCL). Due to the rarity of PBL, the reliable data regarding appropriate treatment of this specific entity is lacking. Because of its high rate risk of central nervous system (CNS) involvement, the CNS prophylaxis may be important. The objective of this study is to clarify the clinical features and treatment outcomes of PBL in the clinical practice.
This is a retrospective study of 17 patients with PBL diagnosed between 2004 and 2014 at Chiba Cancer Center. The patients characteristics including clinical stage, pathology, treatment details and follow up were collected from hospital databases.
The median age was 61(36-84) years. All were female. Eleven, 2 and 4 patients showed stage IE,IIE and IV, respectively. The histological subtype of all 17 cases showed DLBCL. Seven patients underwent surgery. Among 7 patients, 4 and 2 patients received CHOP or R-CHOP therapy after surgery, respectively. All of the 4 patients who received CHOP relapsed and 2 of them died of CNS relapse. Two patients who treated with both R-CHOP and CNS prophylaxis with intrathecal methotrexate injection (IT) are alive without relapse for 50 and 42 months. One patient who received surgery alone because of her elder age is still alive without relapse for 59 months. The other 10 patients were treated with 6 to 8 courses of R-CHOP immediately after the diagnosis. Simultaneously, these 10 patients received CNS prophylaxis with 3 to 4 times of IT during R-CHOP. After a median follow-up of 26 months (4-66), all patients are alive without any relapse.
Our results suggested that the use of rituximab and CNS prophylaxis play an important role in eliminating CNS relapse. In addition, it was seemed that R-CHOP work well in terms of local disease control. From these findings, R-CHOP with CNS prophylaxis consisting with IT has a potential to be a standard care for PBL. At least the surgery is not necessary to cure this special disease entity.
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All authors have declared no conflicts of interest.