Marginal zone lymphoma of the breast-A diminished role for surgery

Primary breast lymphoma by definition is localized to one or both the breasts with or without involvement of regional lymph nodes with no other extra mammary site involvement. Role of surgery in the treatment armamentarium is less well defined.

A 62year old post-menopausal lady was found to have 1.2×1.2×0.6cm mass in the left breast on diagnostic mammography. Core needle biopsy reveals marginal zone lymphoma. Complete staging work-up was performed which was negative for regional spread and metastatic foci. She was diagnosed to have a localized stage IE extra nodal low grade marginal zone lymphoma of the MALT type involving breast. She received radiotherapy to the breast and is in remission at follow up after one year.

Accurate diagnosis requires adequate tissue biopsy either by a core needle or a surgical biopsy for histopathologic evaluation and immunophenotyping. Immunohistochemically the tumors are positive for pan B-cell markers (CD 19, CD 20, CD22, and CD 79a) and lack T cell markers. For stage IE Marginal zone lymphoma radiotherapy alone and for stage IIE combined chemo-radiation offers the best possible control of the disease. Progression free survival for MZL is better than diffuse large B-cell lymphoma.

At present the best evidence support radiotherapy in localized disease and combined chemo-radiation in advanced disease. Role of surgery is minimal and is seen in case of diagnostic dilemma. Rarity of the diagnosis precludes large scale prospective studies making therapeutic decisions difficult and challenging.