Lymphoid and Hematopoietic Neoplasms of the Breast
JUDITH A. FERRY
LYMPHOMAS OF THE BREAST
Primary lymphoma of the breast is generally defined as lymphoma involving one or both breasts with or without ipsilateral axillary lymph node (ALN) involvement, without evidence of disease elsewhere at presentation, in a patient without a history of lymphoma. Tissue should be adequate for pathologic examination, and the lymphoma should be seen in close proximity to mammary tissue,1 although some authorities have not required this last criterion if the diagnostic specimen is a needle biopsy.2 Others have accepted cases as primary if clinically the lesion was a breast mass, even if the biopsy contained fat but no mammary epithelial elements.3 Some investigators also accept cases in which staging reveals more distant lymph node or bone marrow involvement, so long as clinically the primary or major manifestation of the lymphoma is the breast.4
The breast is a very uncommon primary site for lymphoma, possibly correlating with the very sparse endogenous lymphoid tissue in this site. Primary lymphoma of the breast accounts for 0.1% to 0.15%4,5,6,7 of all malignant neoplasms of the breast, for 0.34% to 0.85% of all non-Hodgkin lymphomas (NHLs),4,6,8,9,10 and for less than 2% of all extranodal NHLs.4 Certain types of lymphoma are more likely to involve the breast as primary breast lymphomas, while others are more likely to involve the breast secondarily, that is, secondary to spread from another primary site, in the setting of widespread disease, and/or in the form of a relapse. Different types of lymphoma are discussed separately in the following sections.
Primary Lymphoma of the Breast
Clinical Presentation
Most patients with primary lymphoma of the breast are middle-aged to elderly women, although occasionally young women and, rarely, adolescents are affected,1,5,11,12,13,14 with a median age in the sixth or seventh decade in most series.2,3,6,7,8,9,10,15,16,17,18,19,20,21,22,23 Approximately 2% of primary breast lymphoma patients are males.2,6,7,8,9,10,16,17,18,19,20,21,22,23 Occasionally the disease affects pregnant or lactating women. Rarely patients have had prior breast carcinoma.1
Most patients present with a palpable breast mass with or without ipsilateral axillary lymphadenopathy.2,3,6,8,17,19,22 The lesions are typically painless, but occasionally they are painful.22,24 A few patients have been asymptomatic, and have had the lymphoma detected by mammography. Lymphomas detected initially by routine mammography are typically low-grade lymphomas.6,12,13,15 Constitutional symptoms are uncommon, being found in zero8,15,17,19,25 to 4%21 of patients in different series. In some reports, right-sided lymphoma was more common than left-sided lymphoma.3,26 In 0% to 25% of cases in different series, patients present with bilateral disease. Overall, slightly less than 10% of primary breast lymphomas are bilateral.1,3,5,7,9,10,18,19,21,23,25,27 A few patients have a history of autoimmune disease, diabetes mellitus, or mastitis.4,6,7,17 Several human immunodeficiency virus (HIV)-positive patients have developed lymphoma presenting with involvement of the breast.28 However, most patients have no underlying illness, and specific factors predisposing to lymphoma of the breast have not been identified.2,
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