Teaching Case

A sixty-one year old woman presented to our hospital with palpable lumps on her right breast.

A sixty-one year old woman presented to our hospital with palpable painless lumps on her right breast and axilla. The woman had noticed them 3 months ago but she did not visit her family doctor. Physical examination confirmed the presence of a retroareolar firm mass on her right breast as well as an axillary one. There was no associated skin thickening or nipple retraction. Her past medical history was significant for poorly controlled hypertension and osteoporosis. She denied having any chronic breast disease or family history of breast malignancy. Conventional mammography demonstrated three radiopaque lesions with relatively unsharp contour (fig.1) on the right breast. No micro calcifications or spiculations were found. She underwent ultrasound-guided needle biopsy and the pathological examination of the specimen revealed extensive replacement by MALT lymphoma. Most cells were small cleaved with abundant cytoplasm. Bone marrow biopsy was negative. In order to have the patient completely staged body CT scan was performed . Again the results were negative except for the breast lesions (fig.2). The woman was treated with 6 cycles of chemotherapy and she showed a great deal of improvement (fig.3). Because the lesions were rather extended the chemotherapy treatment is presently followed by adjuvant radiation therapy.

MALT (Mucosa Associated Lymphoid Tissue) lymphomas were first described in the gastrointestinal track by Isaacson and Wright in 1983. Lymphomas arising from MALT became a distinct clinicopathological entity that now includes similar lymphomas at numerous other sites. The median age of presentation of MALT lymphoma is the sixth decade, and the patients often present with extranodal localized disease which may involve a variety of organs including the stomach, small or large bowels, lungs, salivary glands, thyroid, skin, thymus, conjunctiva, tonsils, liver, kidneys, bladder or breast. Breast lymphoma is a rare disease. Both primary and secondary breast involvement have been reported. Primary non-Hodgkin’s lymphoma of the breast is a rare disease, representing only 0.12–0.53% of all reported malignant breast tumours. It is important to determine whether lymphoma of the breast originated in the breast or systemically.Breast MALT lymphomas account for a small fraction of all the MALT lymphomas (1-2%). In most cases, primary breast MALT lymphoma is defined as breast involvement with no other site involved. Most primary breast lymphomas are high-grade malignant neoplasms, mainly large cell and Burkitt’s type. They constitute 0.04%-0.5% of malignant breast neoplasms, 0.07% of all non Hodgkin’s lymphomas (NHL), and 1.7% of extranodal NHL. Low-grade lymphomas of the breast, particularly MALT lymphomas, have been exceedingly rare. The natural history and the optimal management of MALT lymphomas of the breast are not well outlined due to limited experience with this entity. However, these lymphomas are distinct from the non-MALT lymphomas of the breast by virtue of their indolent clinical behavior and prolonged disease-free survival. Many of these patients have no adverse prognostic factors such as bone marrow involvement, high tumor burden, advanced stage, poor performance status, B symptoms, high LDH, high beta-2 microglobulin, low serum albumin or anemia. The low grade type often occurs in the right breast, for unknown reasons. It is commonly in the upper outer quadrant. Radiologic appearance is similar to carcinoma, but calcifications are not usually there. There are two types of mammography findings. 1. A solitary non calcified mass well circumscribed, lobulated or with ill defined margins but not spiculated. 2. Multiple non calcified masses, more common in secondary involvement and only 10% in primary involvement. It is difficult to make any definite conclusions regarding treatment guidelines. However, it is recommended treating patients who have localized disease with excision followed by adjuvant therapy, either radiation or chemotherapy. Patients with disease beyond the breast may be treated with chemotherapy, with local control using excision or radiation. MALT lymphomas of the breast tend to remain localized, and to relapse locally as well. This pattern remains stable independent of treatment (surgery, radiation, chemo singly or in combination).