Burkitt lymphoma (BL) is a highly aggressive non-Hodgkin lymphoma (NHL) of the B-cell. ¹ Three distinct clinical forms of BL are recognized: Endemic, sporadic, and immunodeficiency-associated. Although they have differences in their clinical presentation and geographic distribution, they have similar morphology and immune phenotypic features, and all possess translocation and deregulation of the c-Myc gene which is most commonly resulted from t (8;14), although variant translocations such as t (8;22) and t (2;8) have been described. ² Non-Hodgkin′s lymphoma may originate in, or spread to, any extranodal organ.

Breast lymphoma is a rare disease, either as a primary site or as secondary involvement, representing 0.04-0.5% of malignant breast tumors. ³ It is almost always of non-Hodgkin′s type. Secondary involvement of the breast in patients with diffuse disease is more common. ⁴

Most patients with primary breast lymphoma (PBL) develop a distant disease to other regions. Within the breast, the most common primary lymphomas are B-cell (more rarely T cell) non-Hodgkin′s lymphoma. ³ The specific criteria to the diagnosis of PBL include:

The clinical site of presentation is the breast.

A 23-year-old woman suffered from redness, pruritus and tightness of her breasts in the last trimester of her pregnancy. Symptoms were considered to be related to hormonal change in pregnancy, but her problems aggravated after delivery. She also complained of fever and painful rapid enlargement of her breasts Figure 1. While feeding her child a large amount of serosanguinous fluid drained from her breasts. This time she was admitted in a general hospital under the diagnosis of breast abscess. Figure 1

Burkitt lymphoma involving both breasts

The term "PBL" is used to define malignant lymphoma primarily occurring in the breast in the absence of previously detected lymphoma localizations. Breast lymphoma is a rare disease, either as a primary site or as secondary involvement, representing 0.04-0.5% of malignant breast tumor. ³ PBL of the breast is a rare type of localized NHL, which is usually of the B-cell. It consists only 1% of primary breast tumor, and 2% of extranodal NHL. ⁶ The most frequent histopathologic types are: Diffuse large B-cell lymphoma which accounts for up to 50% of all PBL, follicular lymphoma 15%, mucosa-associated lymphoid tissue (MALT) lymphoma 12.2%, BL and Burkitt-like lymphoma 10.3%. Other histological types of PBL include marginal zone lymphoma, small lymphocytic lymphoma, and anaplastic large cell lymphoma. ⁷ Secondary involvement beyond the breast in patients with diffuse disease is more common. ⁴ , ⁸ The majority of breast lymphoma present as a unilateral painless breast masses in an older woman (average age at diagnosis 55-60) and is usually B-cell non-Hodgkins lymphoma. ⁹ , ¹⁰ , ¹¹ 10% of cases are bilateral, which make it essential to assess contralateral breast. For unknown reasons, the right breast is involved more often than the left. Ipsilateral axillary lymphadenopathy is present in 30-40% of cases. ⁹ , ¹² A less common but distinctive presentation is a young woman in childbearing age who presents during or immediately after pregnancy and is a Burkitt-type lymphoma. ¹² , ¹³ Disease is often bilateral and may clinically mimic inflammatory breast cancer. Breast lymphoma has been reported in men, although they are rare. Systemic "B" symptoms (i.e., fever, weight loss, night sweats) are uncommon. ¹⁴ On physical examination, PBL frequently appears as a benign or less suspicious lesion. ¹⁰ , ¹⁵ PBL usually presents as a single palpable mass that is mobile and contender. Multifocal tumors or diffuse breast involvement also was reported. ¹⁶ Clinical signs of advanced breast malignancy are rare, such as inflammatory changes, nipple involvement, skin retraction, or tumor fixation. ¹² Most patients with primary lymphoma of the breast develop the disease to other sites. Early diagnosis is crucial for outcome. ¹⁷ PBL of the breast remains a diagnosis of exclusion a high index of suspicion and an understanding of the clinical behavior of PBL are necessary for proper patient management, ³ and the diagnosis cannot be made without a complete evaluation. ⁵ If a patient present with a rapidly growing breast tumor, lymphoma should be considered before any surgical intervention is performed. Early decision is vital considering the aggressive nature of the lesion and the prognosis. ³ A distinct mammographic or sonographic pattern has not been reported on the literature because primary lymphoma shows no specific characteristics, which differentiate it from other benign and malignant breast disorders. ⁴ Mammography usually demonstrates a well-circumscribed, uncalcified mass with sharp or minimally irregular margins. ¹⁸ Survival rates reported in PBL varied widely. Some authors have reported 5-year survival rates up to 89% and 50% for stages I and II, respectively. ¹⁹ , ²⁰ Others have reported PBL to have a poor prognosis irrespective of treatment with median survival time as low as 12 months. ²¹ , ²² Local control is excellent with radiotherapy or combined modality treatment but systemic relapses, including in the Central nervous system (CNS), still occur frequently. ²³ In this case, we chose CODOX-M/IVAC regimen and the patient exhibited complete remission. Five month later, while on maintenance therapy, the patient returned with fever and pancytopenia this time in BMA/B showed more than 50% blast, and the patient was treated as acute leukemia with DVP (Daunorubicine, Vincristine, Prednisone) Regimen. This time the patient did not respond to chemotherapy and 1 month later she died from treatment-related complication and sepsis in ICU.

BL of the breast is a rare neoplasm during the pregnancy and postpartum period but highly invasive and associated with poor prognosis if left untreated. PBL is a diagnosis of exclusion. Usually, it takes time to confirm the diagnose BL of breast because patient′s symptoms considered to be related to pregnancy, breast engorgement and hormonal change during the lactation period. Due to highly invasive nature of BL, most patients are diagnosed in high stages or even postmortem. So in any pregnant or lactating woman, who complains of abnormal bilateral breast enlargement, the physician should think about BL or other breast cancers. Diagnostic procedure should be done, and the treatment should start as soon as possible.