scholarly journals Two Cases of The Follicular Lymphoma of The Breast: An Exceedingly Rare Presentation

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S73-S74
Author(s):  
V Kumar ◽  
H Sonani ◽  
T Patel ◽  
J Lam

Abstract Introduction/Objective The primary Non-Hodgkin lymphoma of the breast is a very rare entity, which comprises only less than 0.5% of all breast malignancies. The follicular lymphoma of the breast is still rarer as the most common type is diffuse large B cell lymphoma (DLBCL). We are reporting two cases of follicular lymphoma of the breast. Methods/Case Report Case 1: A 67-year-old female with a history of low-grade follicular lymphoma of the lacrimal gland and cervical lymph node was presented with 1.7 cm irregular soft tissue mass in the upper inner quadrant of left breast identified on CT scan. Microscopic examination showed diffuse infiltration of medium-sized lymphocytes with focal follicular architecture. These lymphocytes were positive for CD20, CD10, BCL2, BCL6, and Ki67 (10%); while negative for CD5, CD3, cyclin D1, and EBER. These findings are consistent with low-grade follicular lymphoma. Subsequently, a biopsy of the right breast mass revealed similar findings. The patient was followed up with a PET scan every six months and doing well. Case 2: A 52-year-old female with a history of low-grade follicular lymphoma of the right hard/soft palate junction was presented with bilateral breast masses identified on mammography. The biopsy was performed and microscopic examination showed vaguely follicular architecture with small to medium-sized lymphocyte infiltration. The flow cytometric analysis revealed CD10 positive B cell population with kappa light chain restriction. Fluorescence in situ hybridization (FISH) analysis showed 96% of cells with BCL2 and BCL6 rearrangement. These findings were consistent with low-grade follicular lymphoma. The patient was started on chemotherapy with good outcomes. Results (if a Case Study enter NA) N/A Conclusion By reviewing two cases, we emphasize the rarity of follicular lymphoma in the breast. The most important differential diagnosis is extranodal marginal zone lymphoma and DLBCL. In absence of flow cytometric analysis FISH analysis for BCL-2 rearrangement or other molecular analysis is key to diagnose follicular lymphoma.

2021 ◽  
pp. 1-9
Author(s):  
Lugos MD ◽  
◽  
Dangana A ◽  
Ntuhun BD ◽  
Oluwatayo BO ◽  
...  

Follicular lymphoma (FL), a non-Hodgkin lymphoma, is an indolent cancer of the B cell lineage that runs a chronic deterioration course that can result in multiple treatment episodes leading to resistance and possible transformation to diffuse large B cell lymphoma. Cytomegalovirus (CMV) reactivation during chemotherapy or after an organ or hematopoietic stem cell transplantation is a major cause of morbidity and mortality. This study tests the hypothesis that some of the heterogeneity of FL might result from chronic infection with Cytomegalovirus (CMV). This research was intended to appraise the impact of CMV infection on the subtypes of T cells in follicular lymphoma patients. We accessed stored peripheral blood mononuclear cells (PMBCs) from patients of known CMV serostatus recruited into an FL clinical trial. We undertook a multicolour flow cytometric analysis of the PBMCs and compared the number of lymphocyte subtypes of CMV-positive and CMV-negative FL patients. Data showed a significant increase in the quantity of terminally differentiated (TEMRA) T cell subsets, including EM3-CD8 (P=0.005), EM3-CD4 (P=0.018), E-CD4 (P=0.029), E-CD8 (P=0.033) and pE2-CD4 (P=0.046) phenotypes, as well as increased NKT cells (P=0.031) among CMV-positive patients compared to the negative group. Our findings support the hypothesis that recurrent infections characterise CMV infection in FL due to accelerated immune senescence and the accumulation of exhausted T cells. Based on the data, a case could be argued for the routine application of CMV screening in FL before treatment with chemo-immunotherapy to implement enhanced infection surveillance in CMV-positive patients. These discoveries can eventually help improve the treatment approaches in the management of FL toward a combinatorial viewpoint for direct cytotoxic and indirect immunomodulatory outlook


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3134-3134
Author(s):  
Ash A. Alizadeh ◽  
Matthew Anderson ◽  
Holbrook E Kohrt ◽  
Ragini M Shyam ◽  
Charles D. Bangs ◽  
...  

Abstract Abstract 3134 Background: Concurrent t(14;18) and 8q24 translocations involving BCL2 and MYC in non-Hodgkin lymphomas (NHL) are rare, but are associated with a inferior overall survival (OS) regardless of the presenting or antecedent histological features (Johnson N, et al. Blood 2009). We sought to confirm these observations in an independent cohort of patients with NHL. Methods: Metaphase karyotypes and/or fluorescence in-situ hybridization (FISH) were used to identify cases of NHL with cytogenetic abnormalities involving 18q21 and 8q24 (BCL2 and MYC). Clinical and cytogenetic characteristics of these patients were assessed for correlations with pathological and clinical variables including outcome. Histological diagnoses were determined according to the 2008 World Health Organization Classification. Overall survival (OS) was calculated from the date a biopsy demonstrated an abnormality involving MYC to the last follow up date or death, as some cases acquired these lesions during their clonal evolution. Result: Among ∼1700 NHL patients diagnosed and/or treated at Stanford University Medical Center on whom cytogenetic studies were routinely performed, we identified 26 patients with evidence for concurrent cytogenetic abnormalities involving BCL2 and MYC. The histological diagnoses at the time of BCL2 and MYC rearrangements were available for 25 of the patients and included follicular lymphoma (FL1-2, n=3; FL3A, n=4), diffuse large B-cell lymphoma (DLBCL, n=2), and B cell lymphoma, unclassifiable, with features intermediate between Burkitt Lymphoma and DLBCL (BCLU, n=16). Cytogenetic analysis revealed that 13/26 cases with both BCL2 and MYC rearrangements had MYC translocations involving the immunoglobulin (Ig) loci. However, in striking contrast to the <10% prevalence of IgL or IgK partners in NHL harboring Ig-MYC, without Ig-BCL2 rearrangements, 9 of these13 cases (69%) involved IgL t(8;22), and only 4 of these 13 cases (31%) involved IgH t(8;14). 9/26 MYC translocations involved various non-Ig loci (35%). One case (1/26) demonstrated amplification of ?both the BCL2 and the MYC loci by FISH, while one case showed trisomy 8 (1/26). Three cases had FISH analysis only, precluding assessment of the MYC rearrangement partner. No correlation between MYC partner and histology was observed, although this analysis was limited by small sample sizes. Non-FL histology correlated with significantly poorer than expected outcome, with a median OS of 4 months compared to 2 y for FL (p=0.003). Interestingly, we found cases with low-grade follicular lymphoma (FL1-2) histology which harbored both BCL2 and MYC rearrangements. To better define the frequency of 8q24 anomalies in an unselected cohort of follicular lymphoma cases, FISH for MYC and BCL2 rearrangements was performed on 192 independent low-grade follicular lymphoma (FL1-2) cases. While a subset of these cases (71) showed the expected frequency of BCL2 rearrangements (72%) by FISH, none showed concomitant 8q24 anomalies by FISH, suggesting that routine FISH analysis of low-grade follicular lymphoma for MYC rearrangements would have a low diagnostic yield. Conclusion: NHL with rearrangements of both BCL2 and MYC are under-recognized due to lack of routine karyotyping or FISH analysis. Comprehensive analysis of BCL2 and MYC status should be performed on all HGBCL in the new WHO (2008) classification, though the low frequency of these lesions among FL tumors precludes a similar conclusion for low grade NHL. Disclosures: No relevant conflicts of interest to declare.


2015 ◽  
Vol 2015 ◽  
pp. 1-9
Author(s):  
Anila Kanna ◽  
Swati Agrawal ◽  
Kumar Jayant ◽  
Varun Kumar Pala ◽  
Mohammad Altujjar ◽  
...  

B cell lymphoma, unclassifiable, with features of diffuse large B cell lymphoma and classical Hodgkin’s lymphoma (BCLu-DLBCL/CHL) is more commonly known as gray zone lymphoma. These cases more often present with mediastinal disease. In this report, we present a very rare case of BCLu-DLBCL/CHL without mediastinal involvement, transformed from follicular lymphoma (FL) to BCLu-DLBCL/CHL. This patient initially presented with a mass in the right neck; biopsy of the lymph node showed predominantly nodular, follicular pattern. Immunohistochemical (IHC) staining of tumor cells expressed positivity for mature B cell markers CD20, CD19, CD10, CD23, CD45, and CD38 but negative for CD5,11c. Hence, diagnosed with FL, he was given rituximab, cyclophosphamide, vincristine, and prednisone (RCVP) regimen, followed by maintenance rituximab. He showed good response. After 2 years, he presented again with a mass in the right side of the neck. Although the needle core biopsy of this mass was suggestive of B cell lymphoma, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. IHC staining expressed positivity for CD20, CD79a, PAX5, and CD15 and CD30 consistent with DLBCL and CHL. He was diagnosed with BCLu-DLBCL/CHL. The patient received “ACVBP” (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation. BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression.


2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Sakshi Kapur ◽  
Miles B. Levin

Double hit B-cell lymphomas are rare tumors that are defined by a chromosomal breakpoint affecting the MYC/8q24 locus in combination with another recurrent breakpoint, mainly a t(14;18)(q32;q21) involving BCL2. These tumors mostly occur in adults and carry a very poor prognosis. Double hit lymphomas can occur de novo, or arise from transformation of follicular lymphoma. We report a case of a 69-year-old female with abdominal distention and progressively worsening weakness over six months. Patient presented with severe hypercalcemia and multiple intra-abdominal/pelvic masses. Histopathology results of the abdominal mass were compatible with a double hit B-cell lymphoma. However, bone marrow biopsy results showed a low grade follicular lymphoma, thus suggesting peripheral transformation of follicular lymphoma to double hit B-cell lymphoma. Patient was transferred to a tertiary care center and was started on combination chemotherapy (EPOCH: doxorubicin, etoposide, vincristine, cyclophosphamide, and prednisone). Our paper highlights not only transformation of follicular lymphoma to double hit B-cell lymphoma and the challenges encountered in diagnosing and treating these aggressive tumors, but also the association of new onset/worsening hypercalcemia in such patients.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2402-2402
Author(s):  
Roberta Sciarra ◽  
Caterina Cristinelli ◽  
Michele Merli ◽  
Marco Lucioni ◽  
Silvia Zibellini ◽  
...  

Abstract BACKGROUND. HCV-positive DLBCL has distinct clinical and pathologic characteristics compared to its negative counterpart: patients (pts) are usually older with more frequent splenic and extranodal involvement and elevated LDH. Differently from its clinical hallmarks, the molecular landscape of this pathological entity has been scarcely outlined. METHODS. In this bicentric study, we investigated the clinical and molecular features and outcome of 54 pts with HCV-positive DLBCL. Targeted next generation sequencing (NGS) was performed on DNA extracted from formalin-fixed paraffin-embedded tissue biopsies. A core panel probes covering coding exons from 184 genes frequently mutated in mature B cell neoplasms was designed using IDT tool and libraries were prepared using Illumina DNA-prep-with enrichment. Sequencing was performed on Illumina HiSeq 2500. Cluster analysis was performed using LymphGen tool. We also applied fluorescence in situ hybridization (FISH) for MYC, BCL2 and BCL6. RESULTS. Median age was 71 (33-84; IQR: 61.9-77). Stage was III/IV in 34 pts (63%). Extranodal sites were involved in 21 pts (38%), spleen in 20 pts (37%). LDH was higher than the upper limit in 40 pts (74%). R-IPI was good for 2 pts (4%), intermediate for 24 pts (44%), poor for 28 pts (52%). HPS score was intermediate or high in 33 of 44 assessed pts (75%). A histological low-grade component was identified in 15 pts (27%). Hans algorithm differentiated pts almost equally in GCB (26/50, 52%) and non-GCB (24/50, 48%) subtype. HCV-RNA was detectable in 52 pts (96%) and quantifiable in 43 pts (79%). Of 29 pts assessed, genotype was 1 in 9 (31%), 2 in 16 (55%), 3 in 4 pts (14%). Among 37 pts whose data were available, 11 pts (30%) received direct antiviral agents, 7 pts (19%) received interferon-containing regimen, 19 pts (51%) were not treated for HCV. Twenty-seven pts (50%) received rituximab-enriched protocols, 23 pts (43%) were treated with chemotherapy alone, 1 pt (2%) with surgery alone, 3 pts (5%) were lost to follow up. With a median follow up of 7.7 years (yrs) (IQR: 4.6-10.6), 5-yrs overall survival (OS) (95%CI) was 49.3% (34.1-62.8%) and 5-yrs progression free survival (PFS) (95%CI) was 39.5% (25.5-53.3%). Median OS and PFS were 4.9 and 3.1 yrs, respectively. FISH analysis showed lack of BCL2 (0/19) and MYC translocations (0/15). BCL6 fusions were found in 76% of pts (16/21). NGS showed mutations in 154 of the 184 analyzed genes. The informativity of the panel was 100% with all pts presenting at least one oncogenic variant. Gene mutation frequencies are presented in Fig. 1. The median mutation load (MML) was 13 mutated genes per case (2-32; IQR: 9-16). Most frequently mutated genes were the epigenetic regulators KMT2D, mutated in 23 pts (42.6%), and SETD1B, mutated in 17 pts (31.5%). FAS, PM1 and RERE were mutated in 15 pts each (27.8%). TBL1XR1, BCL11A and SGK1 were mutated in 14 (26%), 13 (24%) and 12 pts (22%), respectively. Considering genes in their specific pathway, 94% of pts harbored mutations in genes involved in epigenetic regulation (MML: 3; range 1-7; IQR: 1.25-4), 90% of pts in apoptosis-related genes (MML: 2; 1-7; IQR: 1-3) and 77% of pts in genes belonging to BCR/NFkB signaling pathway (MML: 2; 1-7; IQR: 1-3). Of note, 56% of pts carried mutations in genes related to immune regulation (MML: 1.7; 1-5; IQR: 1-2) and 25% of pts had mutations within the NOTCH pathway (MML: 1.2; range 1-2). Via the LymphGen 1.0 tool, we classified 26 pts (48%) into 4 genetic clusters: BN2 (11/26, 42%), ST2 (8/26, 31%), MCD (4/26, 15%), EZB (3/26, 12%). Twenty-eight pts (52%) were classified as "others". Among those belonging to BN2 cluster, 7 pts (64%) had a histologically confirmed transformed DLBCL. No significant differences in terms of OS and PFS were identified according to cluster subgroups. CONCLUSIONS. The prevalence of the BN2 cluster and enrichment of mutations of genes involved in NOTCH pathway seem to indicate a preferential marginal-zone origin in HCV-positive DLBCL. In addition, our data confirm the absence of BCL2 translocation in this subset of DLBCL and show a high prevalence of mutated genes within the epigenetic and immune regulation pathways in HCV-positive DLBCL, pointing out their compelling role in the pathogenesis and suggesting potential implications for molecularly targeted therapies. Figure 1 Figure 1. Disclosures Passamonti: Janssen: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; AbbVie: Speakers Bureau; BMS: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Celgene: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novartis: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Arcaini: Celgene: Speakers Bureau; Gilead Sciences: Research Funding; Bayer, Celgene, Gilead Sciences, Roche, Sandoz, Janssen-Cilag, VERASTEM: Consultancy; Celgene, Roche, Janssen-Cilag, Gilead: Other: Travel expenses.


2018 ◽  
Vol 63 (No. 1) ◽  
pp. 40-49
Author(s):  
P. Borska ◽  
R. Husnik ◽  
P. Fictum ◽  
A. Kehl ◽  
L. Leva ◽  
...  

A four-year-old Bullmastiff weighing 44 kg was presented with a 14-day history of weight loss, vomiting and diarrhoea. Abdominal ultrasonography showed the presence of abdominal lymphadenopathy and thickening of the wall of the descending colon. Esophagogastroduodenoscopy and colonoscopy with biopsy were performed. Histological examination revealed a high-grade lymphoblastic lymphoma, flow cytometric analysis detected malignant cells of the immature B phenotype. PCR for antigen receptor rearrangement confirmed IgH monoclonality pointing together with immunophenotyping to B-cell lymphoma. The dog was treated using a multi-agent chemotherapy protocol. The overall survival time was 487 days. This was an unusual case of primary gastrointestinal B-lymphoblastic lymphoma in a dog with survival equivalent to that of the multicentric form.


2004 ◽  
Vol 31 (1) ◽  
pp. 52-56 ◽  
Author(s):  
Yusef M. Al-Marzooq ◽  
Rajan Chopra ◽  
Mohammed Younis ◽  
Abdulrahman S. Al-Mulhim ◽  
Mohammed I. Al-Mommatten ◽  
...  

2019 ◽  
Vol 28 (3) ◽  
pp. 330-335
Author(s):  
Mansoor M. Nasim ◽  
David J. Chalif ◽  
Alexis M. Demopoulos ◽  
Judith Brody ◽  
Rova Lee-Huang ◽  
...  

Low-grade B-cell lymphoma with immunoglobulin ( IG) and interferon regulatory factor 4 ( IRF4) gene rearrangement is extremely rare, with only 4 cases being previously reported. In this article, we report one additional case that arises from the skull and review the literature. The patient was a 69-year-old man who presented with recurrent and disabling vertigo and was found to have a 5.0 × 1.7 cm lesion within the left posterior parietal bone. Histological examination revealed a bone lesion with diffuse lymphoid infiltrate comprising of mostly small lymphocytes with scant cytoplasm, slightly irregular nuclei and inconspicuous nucleoli, and scattered larger cells resembling prolymphocytes and paraimmunoblasts. Immunohistochemical studies showed that the neoplastic cells were positive for CD20, CD79a, PAX5, CD23, CD43, BCL-2, BCL-6, MUM-1, LEF-1, and IgM and negative for CD5, CD10, cyclinD1, SOX11, and IgD. Flow cytometric analysis identified CD5 negative and CD10 negative monoclonal B cells with lambda light chain restriction. Fluorescence in situ hybridization analysis revealed del(13q) abnormality, but was negative for IGH/BCL2, IGH/CCND1, and BIRC3/MALT1 translocations. Next-generation sequencing identified IGK-IRF4 rearrangement and BRD4 E1113 del abnormalities. Given a low clinical stage (IE) of the disease, the patient did not receive additional treatments and was free of disease at 1 year after the diagnosis.


Sign in / Sign up

Export Citation Format

Share Document