1. Introduction

The breast is a rare site of lymphomatous involvement due to the low proportion of lymphoid tissue at this level [1,2]. Breast lymphoma accounts for only approximately 0.04% to 0.7% of all breast cancer cases [3-6]. A broad variety of histologic types have been reported. The majority are B-cell lymphomas, and the most common type is diffuse large B-cell lymphoma [6].

The imaging features of breast lymphoma are generally nonspecific. However, the treatment and prognosis differ from other breast malignancies; Surgery is not often used as a treatment of lymphoma because of the efficacy of chemotherapy, biological therapy and radiotherapy. To avoid unnecessary treatment, clinicians and radiologists should be familiarized with characteristic imaging features of breast lymphoma.

2. Case Reports

2.1 Case 1

A 42-year-old pregnant patient at 36 weeks consults for bilateral mastitis evolving for 2 months not improved by medical treatment. On examination, both breasts were increased in size, swollen, inflammatory and painful associated with bilateral skin thickening. Mammography objectived a totally opaque left breast, an increase in density of the right breast at the level of its anterior and middle two thirds and skin thickening. There were no detected microcalcifications (Figure 1). US revealed multiple confluent hypoechogenic masses in both breasts, irregular in shape, with indistinct margins, hypervascularized in the Doppler study without posterior attenuation. It also revealed skin thickening and multiple left axillary adenopathy (Figure 1). The patient underwent an ultrasound-guided microbiopsy of the masses and a fine-needle aspiration of the left adenopathy. Histopathological and immunohistochemical findings in the tumour and lymph nodes confirmed the diagnosis of a diffuse large B-cell primary breast lymphoma: CD20+, CD3-, cytokeratin negative.

The CT of the thorax and abdomen performed as a part of the extension workup did not reveal any other localization apart from bilateral mammary involvement and left axillary adenopathy (Figure 1). After four courses of chemotherapy, the CT control showed a partial response to treatment with partial regression of the breast infiltration.

Figure 1: Patient with primary breast lymphoma.

A: Mammography: Increase in density of the right breast at the level of its anterior and middle two thirds; B: Ultrasound: hypoechogenic masse with increase of vascularization in the color Doppler study; C: Heteroechogenic left axillary adenopathy; D: CT: Significant increase in the volume of both breasts, diffuse infiltration without any other locations detected.

2.2 Case 2

A 17-year-old woman, without any pathological history, presented for left mastodynia evolving for 8 months associated with alteration of the general state. Physical examination revealed a firm immobile mass in the internal quadrants of the left breast associated with homolateral enlarged axillary lymph nodes. US showed a voluminous mass of the internal left breast quadrants, irregular in shape with microlobulated margins, mixed echogenicity and generalized increase in vascularisation in the color Doppler (Figure 2). Mediolateral oblique mammography views revealed generalized density increase of the left breast associated with skin thickening (Figure 2).

An abdominal US showed hepatic, pancreatic and ovarian masses with intraperitoneal effusion of medium abundance. Core-needle biopsy of the left breast mass revealed a secondary breast localization of a diffuse large B-cell lymphoma. The extension study with CT showed tumor involvement in multiple other locations.

Figure 2: Secondary diffuse large B cell breast lymphoma.

A: Bilateral mediolateral oblique mammography views revealed generalized density increase of the left breast associated with skin thickening; B, B’: US: voluminous mass, irregular in shape with microlobulated margins, mixed echogenicity and generalized increase in vascularization in the color Doppler

2.3 Case 3

A 60-year-old woman, followed since 2018 for Burkitt lymphoma, presented on mammography bilateral breast masses in the right upper external quadrant and left lower internal quadrant with regular shape, circumscribed contours and medium density associated with adenopathies in the left axillary region without any clinical manifestation (Figure 3). US revealed oval-shaped masses with regular contours, heteroechogenic echostructure, increase Doppler vascularization and left axillary adenopathies of the same appearance (Figure 3).

CT showed bilateral nodular lesions with skin thickening of the left breast and homolateral axillary adenopathies (Figure 4). Breast biopsy of the masses was carried out, with anatomopathological diagnosis of secondary breast lymphoma localization.

Figure 3: Adenopathies in the left axillary region without any clinical manifestation.

A, B: Mammography: Both craniocaudal and mediolateral oblique mammograms demonsrate bilateral masses with regular shape, circumscribed contours and medium density associated with adenopathies in the left axillary region; C: US: Breast oval-shaped masse with regular contours, heteroechogenic echostructure and increase Doppler vascularization; D: Axillary US: Left axillary adenopathies of the same appearance of the breast masses.

Figure 4: CT showed bilateral nodular lesions with skin thickening of the left breast and homolateral axillary adenopathies.

Staging CT: Bilateral breast nodular lesions (thick arrow) with skin thickening of the left breast and homolateral axillary adenopathies (thin arrow).

2.4 Case 4

A 33-year-old woman, followed for diffuse large B-cell lymphoma, presented right mastodynia with a swollen and inflammatory breast on examination. Mammography revealed diffuse density increase of the right breast, skin thickening and bilateral axillary adenopathies (Figure 5). US demonstrated multiple masses of the right breast of regular shape, well circumscribed contours, hypoechogenic without posterior attenuation, showing an increase of vascularization in the color Doppler study associated with a significant skin thickening and bilateral axillary nodes (Figure 5). The breast core needle biopsy with anatomopathological study concluded to a secondary lymphomatous breast localization.

Figure 5: Mammography revealed diffuse density increase of the right breast, skin thickening and bilateral axillary adenopathies.

A, B: Cranio caudal and mediolateral oblique mammography showing generalized density increase of the right breast, bilateral skin thickening and axillary adenopathies; C, C’: Ultrasound: Masses with regular shape well circumscribed contours, hypoechogenic without posterior attenuation.

2.5 Case 5

A 62-year-old woman, followed for aggressive follicular lymphoma, presented on computed tomography that was done as part of the surveillance work up, multiples breast masses associated with axillary adenomegaly (Figure 6). Mammography revealed multiple masses in both breasts as well as bilateral axillary nodes. US showed bilateral heteroechogenic masses, mostly hypoechogenic, hypervascularized in the color Doppler (Figure 7).

Figure 6: Computed tomography that was done as part of the surveillance work up, multiples breast masses associated with axillary adenomegaly.

Axial and coronal contrast enhanced CT showing a significant increase in the volume of the right breast, which shows multiple nodular lesions, and skin thickening. Conglomerate of mediastinal adenopathies extending into the supraclavicular and cervical space. Multiple bilateral axillary adenopathies.

Figure 7: US showed bilateral heteroechogenic masses, mostly hypoechogenic, hypervascularized in the color Doppler.

A, B: Mammography: Multiple dense masses of regular shape and circumscribed contours in both breasts and bilateral axillary nodes; C: Ultrasound: Masse, heteroechogenic, mostly hypoechogenic, hypervascularized in the color Doppler associated with skin thickening and dilated dermal lymphatics.

3. Discussion

3.1 Epidemiology

Breast lymphoma occurs almost exclusively in women [1, 7]. It represents approximatly 2% of all extranodal malignant lymphomas [1, 8] and less than 0.7% of breast malignancy [9]. Primary breast lymphoma is very rare, accounting for only 0.85% to 2% of all extranodal non-Hodgkin's lymphoma. It has been defined as localized involvement of one or both breasts without metastatic disease except ipsilateral axillary nodal involvement [3, 10, 11]. Secondary breast lymphoma (SBL) is more common [3, 12] and falls within the scope of an extra mammary lymphomatous lesion already labelled [1].

The reported median age is in the 5th to 6th decades [3, 9, 13, 14], which is similar to the median age of breast carcinoma. Primary breast lymphoma (PBL) presents however some differences in the age distribution with a bimodal peak: Younger population showing bilateral involvement and older population showing unilateral involvement [15].

3.2 Pathophysiology

Breast lymphoma is subdivided in B-cell or T-cell form, based on the neoplastic cell of origin. The most common type is diffuse large B-cell lymphoma, mostly CD20+ [1, 7, 13] which accounts for more than 80% of the primitive form [7]. B-cell lymphoma may be subdivided into low-grade and high-grade tumors [9]. Less commonly, breast lymphoma can be present in other histopathologic types: follicular lymphoma (15%), MALT lymphoma (12.2%) and Burkitt's lymphoma (10.3%) [7, 16].

Primary breast lymphoma behave similarly to lymphoma of similar histologic types and stages in other sites [6] however PBL in pregnant or recently pregnant woman presents generally high-grade of malignancy, rapidly dissemination and poor prognosis [17].

Breast T-cell lymphoma is rare but his incidence may soon increase: Implant-associated anaplastic large cell lymphoma (ALCL) is emerging as a new clinical entity and both saline and silicone implants have been implicated [9]. They have been shown to induce inflammatory T-cell reaction and lymphoma may occur in the fibrous capsule of the breast implant [3, 9, 18-20]. Some morphologic similarities between lymphoma cells, invasive lobular carcinoma and medullary carcinoma have been reported. Histological misinterpretation may lead to an erroneous diagnosis and incorrect treatment [9].

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