Primary Breast Lymphoma Research Articles

Primary breast T cell lymphoma – a rare case report

Primary breast lymphomas (PBL) are an exceptionally rare form of extranodal non-Hodgkin lymphoma (NHL), occurring in less than 0.5% of all breast malignancies.1 Primary breast T-cell lymphoma (TCL) is an even rarer entity, accounting for only 6% of breast lymphomas. It has an aggressive but poorly understood clinical course, and non-specific features that can mimic inflammatory breast carcinoma (oedema, skin changes and mastalgia).1 Currently, there is no standard treatment protocol for PBL, but a multimodal approach using diagnostic lumpectomy combined with chemotherapy and/or radiation therapy is recommended, versus radical mastectomy alone with no overall survival benefit and higher all-cause mortality rates.2 We present a case of a 64 year old female with a 12 month history of right breast pain with skin changes. Clinical and radiological examination (mammogram and ultrasound) revealed no focal lesion or lymphadenopathy. Multiple biopsies showed no evidence of malignancy. The patient was also treated with IV antibiotics with no improvement. Due to persistent symptoms and concern of inflammatory breast carcinoma, a mastectomy was performed. Haematoxylin and eosin (H&E) stained sections and molecular testing (immunohistochemistry and T cell gene rearrangement) confirmed primary breast T cell lymphoma.

Primary Non-Hodgkin’s Lymphoma of Breast Masquerading as Inflammatory Breast Cancer Clinically in a Patient on TNF Alpha Inhibitors

Background: Primary non-Hodgkin’s lymphoma of the breast is an aggressive and rare disease with DLBCL (Diffuse Large B Cell Lymphoma) being the most common variant followed by Marginal zone lymphoma (MZL), follicular lymphoma (FL) and Burkitt’s lymphoma. Out of these, MZL and FL are less aggressive variants unless they undergo transformation. Primary breast lymphoma can mimic inflammatory breast cancer clinically due to peau d’orange appearance of the overlying skin. Association of primary breast lymphoma with TNF alpha inhibitors is rare but has been reported in the literature. Case presentation: We present one such case where a 56-year-old lady presented with a breast lump and nipple retraction in her right breast for 6 weeks. The patient had a history of psoriatic arthritis and had been on Disease modifying anti-rheumatic drugs (DMARDS) for six years. TNF alpha inhibitor was started 3.5 years ago. Clinically, the case was highly suspicious for inflammatory breast cancer. Bilateral mammogram revealed 80mm irregular density in the right retro areolar region of breast; however, no discrete mass was identified on ultrasound examination. Core biopsy showed extranodal marginal zone lymphoma with features of impending transformation. The neoplastic cells were positive for CD45, CD20, PAX5 and BCL6. These cells were negative for AE1/AE3, CD5, CD10, MUM1 and cyclin D1. The patient was then started on R-CHOP (rituximab-cyclophosphamide-hydroxydaunorubicin-oncovin-prednisone) chemotherapy regime for six cycles. TNF alpha inhibitor was stopped. The patient tolerated the chemotherapy regime well and no flare ups of psoriasis were noted. Conclusion: There was rapid progression of breast tumour within a span of 6 weeks in a patient with no family history, no systemic lymphadenopathy and in a background of TNF alpha inhibitors. Based on these findings and the published literature, a probable association of primary breast lymphomas with TNF alpha inhibitors is likely. Although this causal association may be minimal, more research should be done to consolidate the findings.

Primary Non-Hodgkin Lymphoma of Breast in Young Female-A Rare Entity

Primary Non-Hodgkin Lymphoma of Breast in Young Female-A Rare Entity

Primary Non Hodgkin Lymphoma on the left breast of a 53 year old woman: A rare case report

Primary breast lymphoma (PBL) is a rare but distinct subtype of extranodal lymphoma, comprising 0.5% of breast malignancies, approximately 1% of all non-Hodgkin's lymphomas (NHL) and <3% of extranodal lymphomas. Because the cases are rare, the diagnosis is difficult to establish by routine histopathological examination alone. This paper reports a case of a 53-year-old woman, complaining of a lump in the left breast. The lump is said to have been noticed since approximately 2 years ago on the nipple. The patient said that initially the lump was the size of a grape and then realized that it had enlarged since 3 weeks ago with a more chewy texture. Ultrasound of the left mammary, heterogeneous solid lesion with indistinct borders with dilatation of the drawerous duct with the appearance of intraductal calcification filling the superolateral and superomedial quadrants of the left mammary suggestively malignant (BIRADS 5). The microscopic appearance of the FNAB suggested a malignant round cell tumor. Based on the results of the biopsy and immunohistochemical examination, the results were positive CD 45, CD 20 positive, CD 3 negative, and Ki67 positive in 90% of tumor cell nuclei. It was concluded that it was a non-Hodgkin's lymphoma, with a large diffuse pattern. The patient was reported did not survive.

СЛУЧАЙ НЕХОДЖКИНСКОЙ ЛИМФОМЫ МОЛОЧНОЙ ЖЕЛЕЗЫ

Abstract. Primary breast lymphoma is an unusual clinical entity accounting for 0.05-0.53% of all breast neoplasms. The usual presentation includes a painless palpable mass similar to that of breast carcinoma. Diffuse large B-cell lymphoma is the most common identifiable type of plasmablastic lymphoma based on the histopathological examination. We report an unusual case of a 47-year-old Belarusian woman who presented with a 3-month history of a painless mass in the left breast. A biopsy revealed diffuse infiltration of lymphoid cells. The immunohistochemical biomarkers confirmed the diagnosis of a non-Hodgkin's diffuse large B-cell lymphoma. After completing the chemotherapy, the patient is on follow-up, and at 48 months she is alive without disease activity. This case demonstrates the importance of histological verification for treatment. An integrated approach, including the analysis of clinical and anamnestic data, the results of histological and immunohistochemical analysis, allows for modern differential diagnosis of rare breast tumors.

Clinical prognostic risk analysis and progression factor exploration of primary breast lymphoma

ABSTRACT Purpose : A primary breast lymphomais a rare form of extranodal lymphoma type. We aimed to analyze prognosticriskfactors and explore relapse factors in primary breast diffuse large B cell lymphoma (PB-DLBCL). Methods : From November 2003 to September 2020, sixty-three patients from two medical centers newly diagnosed with PB-DLBCL patients were analyzed retrospectively. Results : The median age was 52, and >50% of patients were post-menopausal. The international prognostics index (IPI) (0–1) was mainlyin the low-risk group (84%), and there were four patients with stage IV (6%) who had bilateral breast involvement. With a median follow-up time of 4.92 years (3.17–8.00), five-year overall survival (OS) and progression-free survival (PFS) were 78.9% and 67.1%, respectively. Univariate and multivariate analyses showed that elevated erythrocyte sedimentation rate (ESR) and B symptoms were independent adverse prognostic risk factors for OS, whereas bilateral breast involvement was unfavorable for PFS. Disease recurrence and relapse occurred in 40% (25/63) patients, mainly in the breast, followed by the central nervous system (CNS) and skin/soft tissue. Conclusion : This is the first study to explore the prognostic risk factors and relapse factorsof PB-DLBCL in a relatively large Chinese PBL cohort. Local breast and CNS recurrence after standard R-CHOP treatment were the main issues we are facing now.

Combination of Baseline Total Metabolic Tumor Volume Measured on FDG-PET/CT, ECOG Performance Status and β2-Microglobulin Have a Robust Predictive Value in Patients with Primary Breast Lymphoma

Combination of Baseline Total Metabolic Tumor Volume Measured on FDG-PET/CT, ECOG Performance Status and β2-Microglobulin Have a Robust Predictive Value in Patients with Primary Breast Lymphoma

The prognostic value of IPI in patients with primary breast lymphoma, a multicenter retrospective study

BackgroundDue to the rarity of PBL and the lack of large-scale studies, the prognostic value of IPI in PBL was controversial. Especially in the rituximab era, the ability of IPI to stratify prognosis in patients receiving immunochemotherapy was severely reduced. Then revised IPI (R-IPI) and National Comprehensive Cancer Network IPI (NCCN-IPI) were introduced. The present study aimed to evaluate the prognostic value of IPI and the other IPIs in patients with PBL in a Chinese population.MethodsWe performed a multicenter retrospective study of 71 patients with PBL from 3 institutions in China. The Kaplan–Meier method and log-rank tests were used for the survival analysis. Cox regression analysis was performed to evaluate the prognostic factors. Subgroup analysis was performed to assess the prognostic significance of IPI scores, R-IPI scores, and NCCN-IPI scores.ResultsThe median follow-up was 4.7 years (0.7–21.8 years). The 5-year progression-free survival (PFS) and overall survival (OS) rates were 90.2% and 96.3%. In the multivariate analysis, only IPI scores and radiotherapy were significantly associated with OS and PFS (P < 0.05). Applying the R-IPI in our patient cohort indicates a significant difference in PFS between the two groups of R-IPI (P = 0.034) but not for OS (P = 0.072). And the NCCN-IPI was prognostic for OS (P = 0.025) but not for PFS (P = 0.066). Subgroup analyses of IPI showed that survival analysis of IPI scores for the PFS and OS of patients using rituximab were not significantly different (P > 0.05).ConclusionsOur study confirms the prognostic value of IPI in patients with PBL, but the predictive value of IPI proved to be relatively low with the addition of the rituximab. The R-IPI and NCCN-IPI can accurately assess the high and low-risk groups of PBL patients but were insufficient to evaluate the intermediate risk group.

Current Understanding of Breast Implant-Associated Anaplastic Large Cell Lymphoma

Every year, breast implants are inserted worldwide for reconstructive or aesthetic reasons. Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rather uncommon type of T cell lymphoma that is positive for the CD30 biomarker. Despite being far more common than other primary breast lymphomas, BIA-ALCL has a very low incidence. Textured types of implants have been linked to almost all cases. The majority of patients have a favorable prognosis after the removal of implants and capsules. Nevertheless, the chance of a fatal outcome is higher with capsular invasion and tumor bulk. Although the exact cause of BIA-ALCL is unknown, it has been suggested that persistent infections or toxins related to the implants may play a role. Therefore, physicians must be aware of breast implants' rare but potentially significant side effects. Before surgery, patients with verified instances should be directed to a breast medical oncologist or lymphoma specialist for oncologic assessment. Total en-bloc capsulectomy, a surgical procedure that removes the implant and the surrounding capsule, is usually adequate. In other cases, a late diagnosis necessitates more invasive surgery and systemic therapies, which, while typically effective, have been linked to poor outcomes and even fatalities. Since it is a recently described entity and the "denominator" (i.e., the total number of breast implant procedures) is unknown, it is challenging to determine the risk of development. In this review, we hope to emphasize the elements of etiology, demography, clinical features, and current treatment approaches for BIA-ALCL. In doing so, we hope to increase the medical professional's knowledge of the recognition and treatment of BIA-ALCL.

Primary Lymphoma of Breast: A Population-Base Outcome Analysis.

The majority of neoplasms of the breast are derived from epithelial components and give rise to carcinoma, namely invasive ductal and lobular carcinoma of the breast. Unlike carcinomas, primary hematolymphoid malignancies of the breast are a rare group of malignant neoplasms. Due to their rarity, these patients' epidemiological features and outcomes have not been studied well. A few limited case series and case reports suggest that this group of heterogeneous neoplasms has female predominance and poor prognosis. However, no systematic study exists to date. In order to bridge this knowledge gap, the National Cancer Institute's Surveillance, Epidemiology, and End Results databases have been quarried and analyzed to investigate the epidemiological and outcome features of primary hematolymphoid malignancies of the breast. This study is one of the first efforts to establish a systematic understanding of the demographic characteristics and the survival features of this rare group of malignancies.

Bilateral primary breast lymphoma in a pregnant woman: a case report and literature review

Bilateral primary breast lymphoma in a pregnant woman: a case report and literature review

"Primary Peripheral T-Cell Lymphoma of Breast in Male Patient: A Case Report and Literature Review"

"Primary Peripheral T-Cell Lymphoma of Breast in Male Patient: A Case Report and Literature Review"

Reports of primary and secondary breast lymphoma: our experience in King Chulalongkorn Memorial Hospital

We report four cases of primary breast lymphoma (PBL) and five cases of secondary breast lymphoma (SBL). We illustrate imaging findings in various imaging modalities including mammography, ultrasonography (US), magnetic resonance imaging (MRI) and positron emission tomography (PET) as well as review literature concerning this uncommon condition.

Primary breast lymphoma in males: Incidence, demographics, prognostic factors, survival, and comparisons with females.

BackgroundPrimary breast lymphoma (PBL) is a rare disease condition and is mainly observed in females. No male PBL cohorts were reported previously. This study aims to investigate the incidence, clinical characteristics, prognostic factors, and survival outcomes among male PBL patients and also to perform comparisons between males and females.MethodsPatients diagnosed with PBL between 2000 and 2019 from the Surveillance, Epidemiology, and End Results (SEER) database were identified. Age-adjusted incidence rates were calculated by year and age for trend analysis. Univariate and multivariate Cox hazard proportional regression analyses were performed to identify prognostic factors. Survival comparisons were conducted using the Kaplan–Meier method and the log-rank test. The propensity matching score (PSM) method was used to balance demographics.ResultsThe incidence rate of 122 male PBL patients diagnosed in the period of 2000 to 2019 was 0.169 (95% CI: 0.140–2.203) per million persons, which was much lower than that of 2,543 females (1.59, 95% CI: 1.53–1.65). Clinical demographics were similar between females and males, except for lymphoma subtype distribution (P = 0.025). A higher age [hazard ratio (HR) = 1.08, 95% CI: 1.05–1.12, P < 0.001] and not receiving radiotherapy (receiving vs. not receiving: HR = 0.41, 95% CI: 0.21–0.78, P = 0.007) were significant risk factors associated with overall survival (OS) in males. Radiotherapy (OS: P = 0.023) can offer benefits in OS. Using the PSM method, we also revealed that male PBL patients had significantly worse OS and cancer-specific survival rates than females.ConclusionsThis study first analyzed male patients with PBL involving incidence, clinical characteristics, and survival data. Sex disparity was also observed in the survival outcome of the disease.

Genomic Mutation Landscape of Primary Breast Lymphoma: Next-Generation Sequencing Analysis.

Primary breast lymphoma (PBL) is a rare subtype of non-Hodgkin's lymphoma (NHL) with rapid progression and high risk of central nervous system metastasis. We have investigated 40 PBL patients retrospectively, and 16 of them were sequenced by a target panel of 112 genes related with lymphoma. Next-generation sequencing (NGS) identified 203 mutations spanning 35 genes and revealed seven potential protein-changing genes (PIM1, MYD88, DTX1, CD79B, KMT2D, TNFAIP3, and ITPKB) with high frequency, referring crucial roles in lymphomagenesis. Our result suggested that PIM1 mutation is correlated with the age and pathological type of PBL patients. Gene TNFAIP3 and KMT2D mutation is only related to the pathological type and primary site, respectively. These high-mutant genes detected in PBL indicated a tendency to shorten overall survival (OS) and progression-free survival (PFS), which may lead to poor prognosis. Furthermore, the nuclear factor kappa-B (NF-κB) pathway and related regulatory factors are essential for the development of targeted therapy as well.

Patterns of treatment and their outcomes in primary breast lymphoma; a comprehensive population-based analysis.

Primary breast lymphoma (PBL) is managed differently among centers, using surgery, systemic therapy and/or radiation. With data derived from the National Cancer Database (NCDB), we aim to describe treatments utilized in the United States, estimate the overall survival (OS) of different therapeutic modalities and determine the role of systemic therapy in patients with PBL. We conducted a retrospective cohort study using de-identified data from the NCDB. The NCDB provided records of 4616 patients diagnosed with PBL between 2004and 2015. We excluded patients diagnosed with HIV, with no survival data, not treated in the reporting facility, without histologic confirmation, with stage III/ IV disease and for whom surgery, radiation, or systemic therapy was contraindicated. Both propensity score weighting and Cox models were used to obtain adjusted estimates. Based on histopathology, PBL was classified into indolent (I-PBL) and aggressive (A-PBL). In a sample size of 2063 PBL patients, the median age was 67years (interquartile range (IQR): 57-78), and 97% were females. In 1027 patients with I-PBL, the median follow-up was 66months (95% confidence interval (CI): 32.6-107.2) and 60% of patients had extranodal marginal zone subtype. Systemic therapy did not improve adjusted-OS (median: 154 vs. 143months, P = 0.36) (Hazard ratio (HR): 0.86, 95% CI: 0.60-1.25, P = 0.42). The treatment arms associated with the highest adjusted 5-year OS were as follows: radiation (85%), surgery (79%), systemic & radiation (87%) and radiation & surgery (87%) (P = 0.9). In 1036 patients with A-PBL, the median follow-up was 67.4months (95% CI: 35.9-105), and 87% of patients had diffuse large B-cell subtype. Patients with A-PBL who received systemic therapy had an improved adjusted-OS (median: 115 vs. 72months, P < 0.01) (HR: 0.45, 95% CI: 0.38-0.53, P < 0.001). The treatment arms associated with the highest adjusted 5-year OS were: systemic (69%), systemic & radiation (77%), systemic & radiation & surgery (79%) and systemic & surgery (79%) (P = 0.4). Systemic therapy used as first-line treatment is essential in A-PBL. Local therapy in the I-PBL using surgery and/or radiation is effective in long-term disease control. There is significant variation in front-line treatment modalities utilized in PBL across the US, many associated with similar outcomes.

Poor Prognosis of Diffuse Large B-Cell Lymphoma of the Breast: The Role of Dose-Dense Chemotherapy

Background: The aim of the study was to define if the use of an intensive regimen (dose-dense) of chemotherapy may improve response rate and outcome in patients with primary breast lymphoma (PBL), whose is considered as lymphoma presentation with an worse prognosis. Methods: Patients with pathological confirmed diagnosis of diffuse large B-cell lymphoma with clinical presentation on breast (PBL) , &gt; 18 years old, stage I and II, negative for immunodeficient syndrome, hepatitis A and B, previously untreated , with non-germinal center cell, were included in a open label clinical trial, phase II, were allocated to received an dose dense regimen of CHOP (cyclophosphamide , doxorubicine, vincristine and prednisone) Results: Between July 2006 to December 2016, seventy eight patients were enrolled in the study. Complete response was achieved in 76 (94.4%), patients. Five patients relapse , thus progression free survival at 5 years was 94.0% (95 % Confidence interval (CI): 87% to 98%%): three patients achieved a second response and are alive, overall survival at 5-years was : 95.3 (96%CI: 89% to 102%).Relapse at central nervous system has not been observed. Severe granulocytopenia was observed in 42.8 (93.0%) cycles, but no death associated to treatment were observed. Conclusion: The use a dose-dense regimen of chemotherapy improve outcome in this setting of patients; although severe acute toxicities were frequent , it well controlled. Relapse in central nervous system has not been observed. Thus, we considered that dose-dense chemotherapy will be employed in this setting of patients.

A Comprehensive Model for Diagnosis of Primary Breast Lymphoma Differentiated From Breast Cancer and Prognosis Evaluation of Surgical Treatment.

BackgroundThe objective of this work was to discriminate between primary breast lymphoma (PBL) and breast cancer by systematically analyzing clinical characteristics, laboratory examination results, ultrasound features, and mammography findings to establish a diagnostic model for PBL and to analyze the influence of surgical treatment on the prognosis of PBL patients.MethodWe analyzed 20 PBL and 70 breast cancer patients treated during the same period by comparing several characteristics: clinical features, such as age, tumor position, and breast complaints; laboratory examination findings, such as the lactate dehydrogenase (LDH) level, and imaging features such as the maximum diameter, shape, margins, aspect ratio, and calcification of the mass and axillary lymph node involvement. A diagnostic model was then developed using logistic regression analysis. The impact of surgery on the prognosis of PBL patients was assessed through Kaplan–Meier survival analysis.ResultBreast cancer and PBL could be distinguished based on imaging features, including the maximum diameter, shape, margin, and calcification of the mass, and lymph node involvement (P < 0.05). There were no significant differences between PBL and breast cancer patients in terms of clinical features, or the LDH level. The area under the receiver operating characteristic curve was 0.821. The log-rank test showed that surgery had no significant influence on the prognosis of PBL patients.ConclusionUltrasound and mammography are the most useful methods for detecting malignant breast tumors. Compared with breast cancer tumors, breast lymphoma tumors are larger with a more regular shape and less calcification and are often accompanied by axillary lymph node involvement. Patients with a breast malignancy should not undergo surgical excision without an accurate diagnosis.

Bilateral Myeloid Sarcoma of the Breast: A Case Report With Radiological and Pathological Correlation

Myeloid sarcoma is a solid extramedullary mass of immature myeloid cells, often in patients with myeloid leukemia. Myeloid sarcoma of the breast is extremely uncommon, and bilateral involvement is even rarer. Myeloid sarcoma of the breast can mimic primary breast cancer, lymphoma, and other neoplasms. Differentiation between myeloid sarcoma and primary breast malignancy is imperative, as management and treatment are drastically different. We present a case of myeloid sarcoma of both breasts in a 63-year-old female with relapsed acute myelogenous leukemia (AML), a personal history of ovarian cancer, and a family history of both leukemia and breast cancer. This report highlights the need for high clinical, radiological, and pathological suspicion to diagnose myeloid sarcoma of the breast.

Primary Burkitt lymphoma of breast presented by bilateral breast enlargement

Burkitt lymphoma is a very aggressive B cell lymphoma, which can rarely involve breast tissue. Although it usually affects children, but primary breast lymphomas usually, occur in older age. In this report, we present a rare case of Burkitt lymphoma in a 40-year-old woman primarily presented by bilateral breast enlargement which shortly followed by gastrointestinal and bone marrow involvement.