Primary Non-Hodgkin’s Lymphoma of Breast Masquerading as Inflammatory Breast Cancer Clinically in a Patient on TNF Alpha Inhibitors

Abstract

Background: Primary non-Hodgkin’s lymphoma of the breast is an aggressive and rare disease with DLBCL (Diffuse Large B Cell Lymphoma) being the most common variant followed by Marginal zone lymphoma (MZL), follicular lymphoma (FL) and Burkitt’s lymphoma. Out of these, MZL and FL are less aggressive variants unless they undergo transformation. Primary breast lymphoma can mimic inflammatory breast cancer clinically due to peau d’orange appearance of the overlying skin. Association of primary breast lymphoma with TNF alpha inhibitors is rare but has been reported in the literature. Case presentation: We present one such case where a 56-year-old lady presented with a breast lump and nipple retraction in her right breast for 6 weeks. The patient had a history of psoriatic arthritis and had been on Disease modifying anti-rheumatic drugs (DMARDS) for six years. TNF alpha inhibitor was started 3.5 years ago. Clinically, the case was highly suspicious for inflammatory breast cancer. Bilateral mammogram revealed 80mm irregular density in the right retro areolar region of breast; however, no discrete mass was identified on ultrasound examination. Core biopsy showed extranodal marginal zone lymphoma with features of impending transformation. The neoplastic cells were positive for CD45, CD20, PAX5 and BCL6. These cells were negative for AE1/AE3, CD5, CD10, MUM1 and cyclin D1. The patient was then started on R-CHOP (rituximab-cyclophosphamide-hydroxydaunorubicin-oncovin-prednisone) chemotherapy regime for six cycles. TNF alpha inhibitor was stopped. The patient tolerated the chemotherapy regime well and no flare ups of psoriasis were noted. Conclusion: There was rapid progression of breast tumour within a span of 6 weeks in a patient with no family history, no systemic lymphadenopathy and in a background of TNF alpha inhibitors. Based on these findings and the published literature, a probable association of primary breast lymphomas with TNF alpha inhibitors is likely. Although this causal association may be minimal, more research should be done to consolidate the findings.