Bilateral Primary Marginal Zone Lymphoma of the Breast – A Rare Breast Malignancy

Abstract

Extranodal marginal zone lymphoma (EMZL)presents only rarely within the breast, although the incidence of breast EMZL has increased in the past decade for unclear reasons. Due to its rarity, the etiology, course, and treatment response of this cancer are less studied. Case Report: We present the case of a 64-year-old female who had bilateral diffuse irregularity in a trabecular pattern on screening mammogram. Random ultrasound-guided breast biopsy of the right breast demonstrated an extra-nodal marginal zone Bcell lymphoma. She also had approximately 25% marrow involvement by mucosa-associated lymphoid tissue-type marginal zone lymphoma and splenomegaly. Clinically she remained asymptomatic during a 1-year follow-up. Although she presented with advanced-stage disease involving both breasts, spleen and bone marrow, given her lack of associated symptoms, she was observed with active surveillance. Conclusion: Asymptomatic cases of breast EMZL can be managed with close observation as exemplified by our case.Extranodal marginal zone lymphoma (EMZL), also known as mucosa-associated lymphoid tissue (MALT) lymphoma, arise only rarely within the breast, although the incidence of breast EMZL has increased in the past decade for unclear reasons. Due to its rarity, the etiology, course, and treatment response of this cancer are less well-studied. Here we present a case of bilateral primary breast EMZL. Patient consent was acquired for reporting this case.

A rare case of synchronous mucosa-associated lymphoid tissue (MALT) lymphoma involving bilateral breasts and subcutaneous tissues

“…However, most relapses are responsive to systemic treatments. Asymptomatic cases of advanced stage disease may be managed with close observation or, alternatively, with chemotherapy, as in the case of our patient [ 3 , 13 ]. The rarity of MALT lymphoma precludes large-scale prospective studies evaluating treatment, clinical course, and long-term outcomes, and creates challenges in therapeutic decision-making.…”

Primary Lymphomas of the Breast: A Review

“… 17 Although PB-MZL can present as a painful palpable mass, 22 many cases are asymptomatic and are only identified as focal asymmetries on mammogram and/or ultrasound. 23 , 24 …”

“…It presents histologically as a vaguely nodular growth pattern ( Figure 3 A) of small atypical cells with moderately abundant pale cytoplasm and irregularly shaped nuclei, which are known as monocytoid lymphocytes 22 ( Figure 3 B). Immunohistochemically, the defining feature of PB-MZL is the almost exclusive absence of CD10; PB-MZL stains positive for CD20 and BCL2 and has a low Ki67 proliferation index 22 , 23 ( Table 2 ). However, CD21, CD35 or CD23 highlight underlying remnants of follicular dendritic cell mesh-works suggestive of adjacent germinal centres ( Figure 3 C).…”

“…However, the authors noted that irradiation and/or rituximab would be considered for this patient if local and/or systemic symptoms should occur. 23 Furthermore, there have been cases where intervention with therapy was required. For example, one case report described a patient who opted for active surveillance but developed multiple new breast masses one year after her initial diagnosis which required the administration of rituximab monotherapy until a complete response was achieved.…”