Varun Gowda,Deepika Shelar,Suresh Harbade,Sarojini Jadhav,Rasika Jadhav
Primary breast lymphoma in pregnancy: A case report
Primary breast lymphoma,Case report,Diagnostic dilemma,Delay in response
Introduction & importance Primary breast lymphoma (PBL) is a rare extra nodal site of non-Hodgkin lymphoma and constitutes 0.4–0.5 % of all breast neoplasms. It mostly affects women. Breast lymphoma is divided into two types: primary and secondary. Primary Breast Lymphoma is when cancerous cells form in both the mammary tissue and the lymphatic system, without any other signs of cancer elsewhere in the body. PBL is usually a non-Hodgkin's B-cell type, with diffuse large B-cell lymphoma (DLBCL) being the most common type. Case presentation Here by described is a case report of 24 years primigravida in third trimester who presented with the complaint of a painful swelling in the left breast, mimicking breast abscess. At the time of presentation, patient denied Incision and Drainage due to consequent risks involved in premature delivery of the baby. Post-delivery patient underwent wound debridement on emergency basis. Biopsies revealed primary breast lymphoma (B-cell). She was referred for chemotherapy. Following which she succumbed after 2 cycles of chemotherapy. Clinical discussion Primary breast lymphoma is considered to be a disease with the potential of systemic diffusion. It typically presents as a painless breast mass in 85 % of cases, but may be mistaken for mastitis during pregnancy. Non-responsive mastitis in pregnant or lactating women should be investigated as it may be a symptom of breast lymphoma. Early detection is crucial due to the aggressive nature of the lesion and prognosis. Conclusion Rapidly progressive, Clinical, and Imaging difficulties for diagnosis, Delay in response to treatment makes us to consider Primary Breast Lymphoma in all patients with breast lump.
Primary breast lymphoma in pregnancy: A case report

Primary breast lymphoma (PBL) is a rare extra nodal site of non-Hodgkin lymphoma and constitutes 0.4–0.5 % of all breast neoplasms. It mostly affects women. Breast lymphoma is divided into two types: primary and secondary. Primary Breast Lymphoma is when cancerous cells form in both the mammary tissue and the lymphatic system, without any other signs of cancer elsewhere in the body. PBL is usually a non-Hodgkin's B-cell type, with diffuse large B-cell lymphoma (DLBCL) being the most common type.

Here by described is a case report of 24 years primigravida in third trimester who presented with the complaint of a painful swelling in the left breast, mimicking breast abscess. At the time of presentation, patient denied Incision and Drainage due to consequent risks involved in premature delivery of the baby. Post-delivery patient underwent wound debridement on emergency basis. Biopsies revealed primary breast lymphoma (B-cell). She was referred for chemotherapy. Following which she succumbed after 2 cycles of chemotherapy.

Primary breast lymphoma is considered to be a disease with the potential of systemic diffusion. It typically presents as a painless breast mass in 85 % of cases, but may be mistaken for mastitis during pregnancy. Non-responsive mastitis in pregnant or lactating women should be investigated as it may be a symptom of breast lymphoma. Early detection is crucial due to the aggressive nature of the lesion and prognosis.

Rapidly progressive, Clinical, and Imaging difficulties for diagnosis, Delay in response to treatment makes us to consider Primary Breast Lymphoma in all patients with breast lump.

Primary breast lymphoma (PBL) is a rare extra nodal site of non-Hodgkin lymphoma and constitutes 0.4–0.5 % of all breast neoplasms [1]. Despite its rarity, it is important to understand and recognize PBL for early diagnosis and treatment. PBL affects women more frequently than men, but can develop in either sex. It is classified into primary and secondary types. Primary Breast Lymphoma is defined as the presence of both mammary tissue and lymphoid infiltrate in close association with no evidence of widespread lymphoma or preceding extra-mammary lymphoma [2], [3]. PBL, which makes up about half of all breast lymphomas, is typically a non-Hodgkin's B-cell type. The most common subtype is diffuse large B-cell lymphoma (DLBCL) [4], [5], [19].

A 24-year-old primigravida in her 9th month of pregnancy, presented with pain in left breast since last 1 month. No history of fever, nipple discharge, loss of weight or loss of appetite. She had no family history of breast malignancy. Right breast was normal on examination. On inspection, left breast was twice larger compared to its counterpart with its nipple displaced inferiorly. Skin over left breast was shiny and stretched. No other skin changes or nipple discharge seen. On palpation, there was tenderness all over left breast with no local rise of temperature. An ill-defined mass of 10 × 9 cm, firm, nodular involving the retro areolar region extending to upper and outer quadrant of left breast. No clinically palpable lymph node. Radiological imaging (Ultrasound and Computerised Tomography) and histopathological investigation (excision biopsy) were performed. The ultrasonography findings revealed a well-defined heteroechoic lesion of size 9.6 × 6.9 × 10 cm (Volume: 330–340 cm3) is noted in retro areolar region of left breast. It shows peripheral vascularity on doppler study and dense echogenic debris within. Features suggestive of partially liquified abscess in retro areolar region of left breast. Histopathology report of excision biopsy (Fig. 1, Fig. 2) showed small round lymphoid cells with round nucleus and scanty cytoplasm arranged diffusely separated by fibrous septae at places suggestive of diffuse small cell lymphoma. Immunohistochemistry profile showed CD 20, BCL-6, MUM1, BCL 2, and CMYC diffusely positive in tumour cells. Staining for CD10 was negative. CD3 and CD5 stains background T-Lymphocytes. The proliferation index was approximately 70–75 %. Final impression of Immunohistochemistry was Diffuse large B-cell lymphoma, non-germinal centre B cell type with double expresser phenotype. In summary, the patient had diffuse large B-cell lymphoma, stage IV B, with bulky disease (multiple enlarged lymph nodes at bilateral axillary (left > right), left supraclavicular, cervical, mediastinal, and mesenteric regions) and multiple extra nodal localization (bilateral breast, paravertebral, thyroid, and liver) (Fig. 5, Fig. 6, Fig. 7, Fig. 8, Fig. 9, Fig. 10). This patient was referred to cancer hospital, where she was advised to receive 4 cycles of chemotherapy with R-CHOP regimen included rituximab, cyclophosphamide, vincristine, and Prednisone. Patient didn't survive this disseminated disease, she died after completing 2 cycles of Chemotherapy. This Surgical case report has been reported in line with the SCARE criteria.

Primary Breast Lymphoma is defined as ‘any lesion that had an adequate pathological specimen; both mammary tissue and lymphomatous infiltrates in close relation to each other; no evidence of wide spread disease and no prior diagnosis of extra mammary lymphoma’ [2]. Primary breast lymphoma (PBL) remains an infrequent neoplasm, surgeons should be aware of this entity in order to distinguish its clinical presentation, management, and prognosis from breast carcinoma cases [7]. It is uncommon in men and most frequently (95 %–100 %) reported among female patients [8]. It is more common in single-breast, particularly right side's upper quadrant [9], [10]. The reason for this is unknown, however in our study patient presented as left breast swelling which later spread to involve both breasts. It is vital to evaluate the contralateral breast since 10 % of cases are bilateral, which are more common during pregnancy or the postpartum period and are likely caused by increased proliferation [11]. Primary breast lymphoma is considered to be a disease with the potential of systemic diffusion [12]. The vast majority (85 %) of PBL patients present with a painless breast mass [6]. It may, however, mimic mastitis when it manifests during pregnancy, as it did in this woman [13]. Though 55 years is the median age at diagnosis (17–90 years). In 15 % of cases, PBL occurs in young women of childbearing age (median age, 28 years), during pregnancy or at lactation [3]. In 2008, Shulman et al. reported a case of non-Hodgkin's lymphoma of the breast who presented at 30 weeks gestation with the complaint of painful swelling of the breast for two to three days [13]. PBL can be classified based on the presenting symptoms as type A (atypical symptoms) and type B (fever, night sweats, 10 % weight loss within half a year) [8] In this patient, tumour presented as a painful lump in the breast during third trimester of pregnancy mimicking lactational mastitis. Mastitis is frequently accompanied with systemic infection symptoms, which were evident in this patient and supported the initial diagnosis [13]. Patient underwent debridement (Fig. 3) and biopsy was sent which showed large B-cell lymphoma (non-germinal centre type). However, the lower incidence and atypical clinical manifestations makes it difficult to discriminate from lactational mastitis without confirmation by pathological biopsy. After initial surgical intervention breast did not return to a state of normal pregnancy and there was rapid increase in the breast mass size and development of axillary and cervical lymphadenopathy and swelling in contralateral breast (Fig. 4). The role of progesterone and its immunosuppressive action during pregnancy is considered to predispose the rapid progression of lymphomas [12], [13], [14]. Which is evident from this case that in a span of 3 months patient developed systemic manifestations. Over the past 50 years, there has been a change in PBL treatments. However, given to the rarity of this type of cancer, there isn't currently a standard treatment for PBL in the literature [15]. Surgery such as mastectomy or wide local excision is usually not indicated, as often these tumours respond very well to radiotherapy and systemic chemotherapy [6]. As PBL may be grouped with large B-cell lymphoma histologically [3], [16], the treatment adopted by most haematologists is R-CHOP i.e. rituximab, cyclophosphamide, doxorubicin, vincristine with or without radiotherapy [15]. However, there have only been a few reports of patients with PBL who were treated successfully with R-CHOP with and without radiotherapy. This 24-year-old postpartum patient did not survive this disseminated disease even after receiving 2 cycles of R-CHOP. It is essential to evaluate what could have been done differently and how to manage such cases in future. One significant aspect to consider is the delayed diagnosis and treatment of the patient's lymphoma until after giving birth. In retrospect, an early diagnosis and prompt initiation of treatment could have potentially improved the patient's prognosis. A core needle biopsy at the time of presentation would have likely provided the diagnosis with minimal risk to the patient and her unborn child. This could have been a safer and more efficient diagnostic approach compared to a surgical biopsy, which may have posed a greater risk to the patient and her unborn child. Another consideration is the potential role of early induction of birth in the 9th month of pregnancy, which could have allowed the patient to start systemic therapy for her lymphoma earlier. Nevertheless, careful consideration should be given to the risks of prematurity and other potential complications for the unborn child. It is worth considering whether the patient could have started her chemotherapy during her 3rd trimester of pregnancy, as there are chemotherapeutic regimens that can be safely administered to pregnant women after the end of the first trimester. If deemed necessary, the patient's medical team could have considered initiating chemotherapy during pregnancy, taking into account the potential benefits and risks to both the mother and the unborn child. The choice of chemotherapy regimen should consider the potential risks to the foetus and the effectiveness of the treatment for the patient. While cyclophosphamide, doxorubicin, and vincristine have been safely used during pregnancy, some chemotherapy drugs, such as methotrexate and bleomycin, are contraindicated during pregnancy due to their teratogenic effects.

In conclusion, the case emphasizes the crucial role of timely and accurate diagnosis and treatment of cancer during pregnancy. Collaborative efforts among obstetricians, oncologists, and other specialists are essential for achieving the best possible outcomes for both the mother and foetus. The diagnostic difficulties associated with rapidly progressive, clinically elusive, and challenging-to-diagnose neoplasms, such as Primary Breast Lymphoma, highlight the importance of maintaining a high level of suspicion and an in-depth understanding of the disease's clinical behavior.

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of written consent is available for review by the editor-in-chief of this journal on request.

Ethical approval was provided by authors' institution.

None.

Dr. Deepika Shelar: Data collection, Study concept. Dr. Varun Gowda: Writing the paper. Dr. Rasika Jadhav: Data analysis. Dr. Suresh Harbade: Mentor. Dr. Sarojini Jadhav: Mentor.

Dr. Varun Gowda.

None.

No conflict of interest.