Lymphomatous involvement of the breast is an uncommon occurrence, with an estimated incidence of less than 0.5% of all breast malignancies. 1 Of all extranodal lymphomas, the breast is a site of disease in 0.22% of the patients. 2 Most of these patients have breast involvement caused by metastatic spread of a systemic lymphoma. However, a portion of the patient population have primary breast lymphoma (PBL), with no evidence of disease elsewhere. 3 The true incidence of PBL is unclear, because some authors include patients with locoregional lymph node disease, whereas other reports exclude such patients. 1,4,5 Lamevec and Jancar 5 in 1987 estimated that the number of published reports of PBL was approximately 250 cases.
We herein report a patient with PBL who, after a hiatus of 139 months, had a second PBL develop in the contralateral breast.
A 68-year-old white woman sought treatment in August 1986, with a palpable nodule in the right breast. Family history was negative for malignant disease.
Physical examination showed nothing abnormal except for mild kyphosis. There was no palpable lymphadenopathy. Routine laboratory studies were within normal limits. Spine radiographs showed minimal compression deformities of the eighth and ninth thoracic vertebrae.
An excisional biopsy of the breast mass was performed. The pathologic diagnosis was non-Hodgkin’s lymphoma, B-cell, diffuse large cells. The patient underwent staging procedures that included nuclide scans of bone, liver, and spleen, a total body gallium scan, computerized tomography of the thorax and abdomen, and a bone marrow examination. There was no evidence of disseminated disease.
The patient was referred for radiation therapy. She received 4,500 rad to the right breast and right supraclavicular area during 37 days. There followed a boost of 540 rad to the tumor bed.
Her clinical course in the ensuing 12 years included the onset of hypothyroidism, trauma-related fracture of a femur, and therapy for hypertension.
In March 1999, on routine examination, a 3-cm mass was noted in the lateral aspect of the left breast. An excisional biopsy was performed. The pathologic diagnosis was non-Hodgkin’s lymphoma, B-cell, diffuse large cells. The neoplastic cells, marked with CD20 and common leukocytic antigen (CLA), showed no reactivity to CD3, kappa, and lambda light chains. Comparison of the two tumors was undertaken, and they were deemed to be morphologically identical.
Routine laboratory studies were within normal limits. Staging procedures, as before, were negative for disseminated disease. Chromosome analysis performed on the marrow specimen was normal.
The patient was again referred for radiation therapy. She received a total of 5,040 rad during 40 days. Restaging after 4 months disclosed no evidence of lymphoma.
Non-Hodgkin’s lymphoma may originate in, or spread to, any extranodal organ. As noted before, the breast is a rare site of metastatic disease. Even less frequent is the breast, the organ in which a non-Hodgkin’s lymphoma originates. In the report of Mambo et al., 1 only 14 of 44 patients listed as having breast lymphoma were designated as PBL. Lin et al. 6 noted PBL in 21 of 45 patients with hematopoietic breast neoplasms.
The histology of PBL is primarily B-cell, diffuse large cell, although other histologies, and rare cases of T-cell phenotype have been reported. 6,7 Usually unilateral at the time of diagnosis, bilateral breast involvement has been described. 1,4,6 Of some interest, the pathology of the latter subgroup may have a more varied histologic type. 8,9
Reports of development of lymphoma in the contralateral breast, after a prolonged hiatus, and without evidence of systemic disease, are few. 1,5,7 Our patient’s contralateral breast lymphoma occurred 139 months after the initial tumor diagnosis. We believe the prolonged interval between lymphomas is suggestive of a second PBL rather than a manifestation of systemic spread of the original tumor.
Because of the paucity of patients with PBL, the optimal treatment for this tumor is uncertain. Occasional patients have been cured by surgery, and both chemotherapy and radiation therapy have eradicated disease. 4,5,7 Equally uncertain is the prognosis of patients with PBL. The median survival in the report by Abbondanzo et al. 4 was 36 months, leading them to conclude that PBL had a poor prognosis. In the article of Cohen et al., 7 50% of the patients had recurrent disease at a median of 28.3 months, but only 25% had died of the disease at the time of their report. Thus, prolonged survival has been documented in a significant portion of patients with PBL.
The patient herein described had bilateral breast lymphoma with an interval of 139 months between tumor appearance. Extensive staging procedures confirmed that each was a PBL. Radiation therapy was the treatment modality. The patient remains disease free.
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