Rare Primary Diffuse Large B-Cell Lymphoma of a Male Breast

Background: Breast lymphomas are typical extranodal types of lymphoma, also known as Extranodal-lymphoma (ENL), which occur extremely infre-quently, aggregating into a very small proportion of malignant breast tumors. The rarity of breast lymphomas is attributed to the scant lymphoid tissue content of the chest wall. Aims of Study: This case report is aimed at providing an up-to-date review of the literature on breast lymphomas for clinicians to, therefore, consider the possibility of this disease entity while treating a breast mass. Case Presentation: A case was reported of a 52-year man with chief mammary non-Hodgkin breast ENL when fine-needle aspiration cytology (FNAC) was not leading to a firm conclusion or result. Following an inci-sional biopsy, he was found to have a primary breast lymphoma. Later, the patient was diagnosed with the diffuse large B-cell type of lymphoma also known as non-Hodgkin’s Lymphoma (NHL). He had a complete reduction and disappearances of all the signs and symptoms of the disease after a course of neoadjuvant chemotherapy: Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone (CHOP). CONCLUSION: Based on the above case presentation, it is vital for health care professionals and oncologists to recognize the disease by assessing the breast mass accurately with more entities so that proper diagnosis via core biopsy (incisional biopsy) can eliminate the PBL before further treatment is required.


Introduction
Primary breast lymphoma (PBL) is both a typical yet rare clinical entity: a very well-defined subcategory of non-Hodgkin's Lymphoma (NHL). Several reports suggest that PBL represents 0.6% of all malignant breast tumors, 1.2% of all the NHL, and 2.1% of Extranodal-lymphomas (ENL) [1] [2] [3] [4] [5]. Most breast lymphomas are of the non-Hodgkin's type, representing approximately 70% -90% of all PBL, while diffuse large B-cell lymphomas (DLBCL) constitute 46% -71% of all PBL [6]. However, (P-NHL) Primary-NHL is one of the most frequent and hematopoietic tumors of the breast [7] Aims of Study: This case report is aimed at providing an up-to-date review of the literature on breast lymphomas for clinicians to, therefore, consider the possibility of this disease entity while treating a breast mass.

The Case Presentation
A 52-year-old male industrial chemist was admitted to the surgical outpatients' department at a tertiary hospital with a painless left breast lump that had been evident for 8 months and had increased in size progressively. There was a darkening of the overlying skin and no nipple discharge. The patient reported remarkable weight loss and intermittent fevers over the period, but also reported no cancer history in his family. He had self-treated with herbal medications for some months prior to arriving at the tertiary hospital. Physical examination revealed a middle-aged man, who appeared chronically ill and cachectic. The left breast was diffusely enlarged with a ~4 cm × 3 cm mass located in the center portion with a darkening of the overlying skin. He also had discrete tiny ulcers in the nipple-areolar complex oozing a small amount of serosanguinous fluids with a few other lymph nodes such as Axillary lymph nodes. FNAC revealed potential malignancy, but results were inconclusive. He subsequently had an incisional biopsy with a histopathology report, which confirmed PBL distinguished as a large B-cell type of non-Hodgkin's lymphoma. The observational photograph of the index patient is as displayed in Figure 1(a). Figure 1(b) shows the characteristic diffuse lesion as seen in the mammographic study. Figure 2 and

Discussion
The index patient is male with a lesion located on the left breast. This finding concurred with previous reports indicating that both Nodal-Lymphomas (NL) and Extranodal-Lymphomas (ENL) occur more frequently in males, with the involvement of the breast being mainly associated with females [8]. However, this finding contradicts those of other studies which reported that breast PNHL involved only the right side of the breast, and was almost entirely found in female patients. It is unclear why the right side of the breast is the most common site for these [7]. Primary-Lymphomas (PL) of the breast is generally regarded as rare, yet they remain the most frequently occurring hematopoietic breast tumor [7] [9] [10]. Physicians or oncologists treating and dealing with the carcinoma in the breast should endeavor to be fully up-to-date with knowledge regarding this entity to distinguish the signs and symptoms associated with prompt management of the condition. Indeed, the early prognosis of breast carcinoma is important for satisfactory clinical outcomes [10] [11] [12] [13]. In addition, single-breast involvement is more common, particularly in the upper-right portion of the breast [14] [15] [16] [17]. Only approximately 1% -14% of reported primary breast lymphoma affects both sides of the breast (bilateral) disease [18].
There have been many diagnostic criteria of primary-breast lymphoma [15].
In 1972, Wiseman and Liao defined the existence of adequate pathologic specimens as diagnostic criteria for P-NHL. There is also a very close association of the mammary tissue and lymphomatous infiltration, with no previous diagnosis of an extramammary-lymphoma, with possible ipsilateral axillary lymph node involvement [7] [15] [19]. Our patient fulfilled these criteria, and this further supports our conclusion that this was an instance of a primary breast lymphoma (PBL), and those breast-cancers, in general, are not diseases associated with the younger age group. Finally, PBL is most commonly seen as a palpable mass, as shown in our index patient [20] [21].
An open biopsy supported with immunophenotyping proved to be a very reliable confirmatory diagnostic tool. Tru-cut biopsies have equally distinguished the diagnosis of breast-malignancies with a good accuracy rate [22] [23]. Many radiographic detection methods, such as CT, sonography and mammography, are generally nonspecific for PBL [24] [25] [26] [27]. It should be noted that immuno-phenotyping in the diagnosis of PNH-Lymphoma of the breast is crucial, with several reports from the literature confirming that immuno-phenotyping plays a key role in evaluating and diagnosing the disease [28] [29] [30] [31] [32].
In our index patient, the histopathological diagnosis was PBL of the large B-cell type (non-Hodgkin's type). The tumor cells typing were negative for CD3, CD5, CD15, CD30, CK but were positive for CD45, CD20, CD10, and BCL6 (see Several treatment options are available for primary breast lymphoma, such as chemotherapy as the first preference of treatment of primary-BL, and radiation as a second preference, followed by surgery. The above-mentioned preferences can be used alone or in combination [32] [33] [34] [35]. The impact of surgeries as a treatment option for PBL is limited due to surgery requiring adequate tissue for proper diagnostic criteria and classification of the disease [31] [33]. Finally, mastectomy offers no guarantee of preventing recurrence [36]. For Stage I disease, the 5-year survival rate is 89% [35]. Finally, the central nervous system [CNS] is regarded as the most typical site of relapse due to its high level of incidence and involvement based on available reports [38] [39].

Conclusion
Based on the above case presentation, it is obligatory for health care professionals and oncologists to recognize the disease by assessing the breast mass accurately with more entities to provide the possibility of distinguishing the disease via proper diagnosis, including core biopsy (incisional biopsy), and thus eliminate PBL before further treatment is required.

Authors Contributions
All authors have made substantial contributions to conception and design, acquisition of data, and analysis and interpretation of data. All authors were involved in drafting the manuscript and its critical revision. All authors have given Case Reports in Clinical Medicine final approval of the version to be published. Each author has participated sufficiently in the work to take public responsibility for appropriate portions of the content. All authors agree to be accountable for all aspects of the work in terms of ensuring that questions related to the accuracy or integrity of any part of the work were appropriately investigated and resolved.

Ethical Approval
We confirm that no ethical approval is required to publish case reports from our institutions and the collaborative hospital.

Conflicts' of Interest
The authors declare no conflicts of interest regarding the publication of this paper.