Primary Dural Lymphoma Masquerading as a Meningioma: A Rare Clinical Entity

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S114-S114
Author(s):  
Sakshi Sakshi ◽  
Ashish Bains
Keyword(s):  
B Cell ◽  
Dura Mater ◽  
Occipital Lobe ◽  
B Cell Lymphoma ◽  
Mass Effect ◽  
Proliferation Index ◽  
Right Visual Field ◽  
Pcr Analysis ◽  
Low Grade ◽  
Ki 67

Abstract Primary dura mater–based lymphomas are extremely uncommon and when detected are often clinically and radiologically misidentified. As per literature search, they account for <0.1% of all non-Hodgkin lymphomas; however, a precise incidence is unknown since only a few cases have been described in the literature. Here we report a case of a 64-year-old female who presented for evaluation of a newly diagnosed left tentorial tumor, diagnosed as “consistent with meningioma” on imaging studies with significant mass effect on the left occipital lobe and left cerebellum with effacement of the fourth ventricle. She had been experiencing disabling headaches, balance dysfunction, and reduced vision. On examination, the patient had right visual field defect with wide-based gait. No evidence of systemic lymphoma or clinically suspicious lymphadenopathy was documented. An intraoperative consultation revealed sheets of lymphocytes, rather highly concerning for lymphoma. H&E sections showed dense fibroconnective tissue heavily infiltrated by mature small- to intermediate-sized lymphocytes with irregular nuclei and condensed chromatin without a discernible architecture. Only sparse and scattered larger lymphocytes were seen. Flow cytometry identified a subset of B cells with lambda-restricted immunophenotype expressing CD19+, CD20+, CD10+, CD5–, and CD23–. On tissue sections, Ki-67 showed an overall proliferation index of 10% to 20%. However, no residual follicular dendritic cell meshwork was detected by CD21 or CD23. PCR analysis detected clonal B-cell IgH and IgKappa gene rearrangements. A diagnosis of low-grade CD10-positive B-cell lymphoma (likely follicle center-cell origin) was made and the patient was discharged after 3 days of surgery for outpatient follow-up and treatment. In conclusion, low-grade dural-based lymphomas are extremely rare and often misdiagnosed as meningiomas clinically and radiologically. Additionally, it is important distinguish lymphomas of the dura mater, which are excluded from the definition of primary CNS lymphomas and may have a different clinical management and outcome.

Clinical and Biological Features of Primary Testicular B-Cell Lymphoma - a Single-Institution Study of 89 Cases

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 3036-3036
Author(s):  
Sanam Loghavi ◽  
Zijun Yidan Xu-Monette ◽  
Luis Fayad ◽  
Larry W Kwak ◽  
Bouthaina S. Dabaja ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
B Cell ◽  
Cell Lymphoma ◽  
Cell Lineage ◽  
B Cell Lymphoma ◽  
Intrathecal Chemotherapy ◽  
Stage Iii ◽  
Proliferation Index ◽  
Ki 67 ◽  
Testicular Lymphoma

Abstract Context: Primary testicular lymphoma (PTL) is an aggressive form of extranodal lymphoma almost always of B-cell lineage. The data regarding prognostic indicators and appropriate treatment regimens in patients with PTL, especially in the era of rituximab-cyclophosphamide, hydroxydaunorubicin, vincristine and prednisone (R-CHOP) therapy are not well established. Objective: To evaluate the clinicopathologic features and their impact on outcome in patients with primary testicular lymphoma (PTL) of B-cell lineage. Patients and Design: We retrospectively analyzed the characteristics of 89 patients with PTL referred to our institution between September 1, 1998 and May 31, 2014. Follow up data was available for all patients. Detailed staging and therapy information were available for 69 and 63 patients, respectively. Cell of origin classification of DLBCL cases was determined by immunohistochemistry when possible. Results: The median age at diagnosis was 63.1 years (range, 2.9-96.2). Patients presented with stage I (26/66, 40%), stage II (14/66, 21%), stage III (4/66, 1%) and stage IV (25/66, 38%) disease, respectively. Histologic variants included: diffuse large B-cell lymphoma (DLBCL) (n=82), Burkitt lymphoma (BL) (n=2), B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and BL (n=4) and blastoid mantle cell lymphoma (n=1). Twenty-six (40%) patients had involvement of spermatic cord, epididymis and paratesticular tissues and 16 (18%) tumors showed coagulative tumor necrosis. In 31 cases DLBCL was stratified into germinal center B-cell-like (n=16, 52%) and non-germinal center B-cell-like (n=15, 42%). Six of 36 (16%) tumors analyzed showed CD5 expression by immunohistochemistry and/or flow cytometry. The median Ki-67 proliferation index was 80% in 24 cases analyzed (range, 40-100%). Four of 16 cases (25%) analyzed were positive for MYC rearrangement by fluorescence in situ hybridization. Among patients with available data (n=63); all received systemic chemotherapy (41, R-CHOP, 9 CHOP and 13 other regimens) +/- prophylactic intrathecal chemotherapy (n=48) and/or consolidation involved field radiation therapy (n=37, 23 patients with stage I/II and 12 patients with stage III/IV disease). Response to therapy was documented in 61 patients; 85% achieved complete remission, 7% partial remission and 8% had progressive disease (PD). Recurrent and/or PD were seen in 28 of 65 (43%) patients with available data. The most frequent sites of recurrence included lymph nodes (23%), soft tissues (19%), leptomeninges/brain (19%) and contralateral testicle (13%). The median overall survival (OS) and progression free survival (PFS) were 84.7 and 55.7 months, respectively. Advanced Ann Arbor stage (p=0.05, PFS), involvement of ≥2 extranodal sites (p=0.05, OS), elevated serum LDH level (p=0.013 and 0.026, OS and PFS respectively), and International Prognostic Index ≥2 (p=0.04, OS) were significantly associated with poorer outcome. Administration of prophylactic intrathecal chemotherapy (p=0.0017 and 0.012, OS and PFS, respectively) and consolidation radiation therapy (Figure 1, p=0.01 and 0.033, OS and PFS, respectively) were significantly associated with better outcome by univariate analysis. The association of consolidation radiation therapy with outcome was diminished in patients with stage III and IV disease. There was no significant association between different chemotherapy regimens and outcome, although patients who received rituximab showed a trend for better OS and PFS. Microscopic involvement of spermatic cord, epididymis and paratesticular tissue was associated coagulative necrosis (p= 0.04) and poorer outcome (p=0.018 and 0.023, OS and PFS respectively). Non-DLBCL histology type was significantly associated with nodal involvement at presentation (p=0.05). We observed a trend for shorter PFS in patients whose tumors had a Ki-67 proliferation index of ≥90% (p=0.2). There was no significant association between GCB vs. non-GCB subtypes, CD5 expression or MYC gene rearrangement and outcome. Conclusions: Our results indicate that PTL of B-cell lineage represents a distinctive group of extranodal lymphomas with unique clinical, biological and prognostic features. The data further demonstrate the beneficial therapeutic impact of prophylactic intrathecal chemotherapy and consolidation radiation therapy in patients with PTL. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Tissue microarray: A valuable method in diagnosis and prognosis of hematological malignances

2005 ◽  
Vol 13 (3-4) ◽  
pp. 131-135 ◽  
Author(s):  
Goran Marjanovic ◽  
Stefan Dojcinov
Keyword(s):  
B Cell ◽  
Tissue Microarray ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cost Effective ◽  
Lymphocytic Leukemia ◽  
Proliferation Index ◽  
Ki 67 ◽  
Diagnosis And Prognosis ◽  
Valuable Method

BACKGROUND: The novel technology of tissue microarray (TMA) allows rapid and cost-effective analysis of hundreds of markers on the same set of specimens. Limited amount of tissue that could be analyzed and problem of tissue heterogeneity, are the major drawbacks of TMA technique for immunohistochemical characterization of lymphomas. METHODS: In this paper 65 cases of lymphomas were analyzed using TMA with following panel of antibodies: BOB1, Oct2, Bcl2, Bcl6, CD20, CD21, CD23, CD3, CD5, CD10, CD43, CD79a, CD138, Cyclin D1, Ki67, MUM1, Pax5, p53. RESULTS: In 14 patients with diffuse large B-cell lymphoma (DLBCL), 5 were classified as germinative center and 3 as non-germinative center cases according to the Bcl6, CD10, and MUM1 positivity. Other 2 patients were identified as T cell rich B cell lymphoma based on morphology and Oct2 and BOB1 positivity of pleomorphic B lymphocytes. DLBCL with Bcl6+ immunophenotype had better overall survival than Bcl6- cases. All cases of classic mantle cell lymphoma had significantly lower Ki-67 proliferation index than blastoid subtypes. There were 14 cases of chronic lymphocytic leukemia/small cell lymphocytic lymphoma, 6 cases with follicular lymphoma, 5 cases of marginal zone lymphoma, and 7 cases of lymphoplazmacitoid lymphoma. In the indolent lymphoma group, survival of patients with p53+/- was poorer comparing to p53- group. CONCLUSION: We conclude that TMA technique is a valuable method in diagnosis and prognosis of lymphomas, which are considered very heterogeneous group of hematological neoplasms.

scholarly journals Comparison of Ki-67 Labeling Index Patterns of Diffuse Large B-Cell Lymphomas and Burkitt Lymphomas Using Image Analysis: A Multicenter Study

Diagnostics ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 343
Author(s):  
Yosep Chong ◽  
Tae Eun Kim ◽  
Uiju Cho ◽  
Min-Sun Jin ◽  
Kwangil Yim ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Labeling Index ◽  
Proliferation Index ◽  
Epstein Barr Virus Infection ◽  
True Negative ◽  
Ki 67 ◽  
Barr Virus ◽  
Large B Cell

Diffuse large B-cell lymphoma (DLBCL) is the most common high-grade B-cell lymphoma found in Korea; it manifests with a variety of cellular morphologies and a high proliferation index. It is difficult to differentiate between DLBCL and Burkitt lymphoma (BL) based on immunohistochemistry, histology, and Epstein-Barr virus infection status owing to the overlap in findings. In this study, we performed comparative morphometric analysis to understand the proportional difference in Ki-67 staining between DLBCL and BL. We analyzed Ki-67-stained slides of 103 DLBCLs and 29 BLs that were pathologically confirmed using a three-tier classification system (negative, 1+, 2+, and 3+) to compare Ki-67 expression between BL and activated B-cell and germinal center B-cell subtypes of DLBCL and DLBCL with high proliferation indices (>90% of 2+ and 3+ cells). Patients with DLBCL were older than those with BL (62.1 versus 51.0 years). The number and proportion of negative cells (passenger and true negative cells) were significantly lower in BLs than those in DLBCLs (337.4, 5.9% versus 690.3, 12.4%). The number and proportion of 3+ cells were significantly higher in BLs than those in DLBCLs (5213.6, 96.3% versus 3132.4, 62.0%). BLs and DLBCLs with a high proliferation index showed similar results as those between BLs and overall DLBCLs. We were able to differentiate BLs and DLBCLs with 98.1% sensitivity and 100.0% specificity using an optimal cut-off of 97.9% of 2+/3+ Ki-67-positive cells. Thus, the Ki-67 labeling index may be a good differential biomarker for DLBCLs and BLs.

Atypical Plasmablastic Lymphoma Presenting as a Perianal Mass in a 19-Year-Old Male

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S110-S111
Author(s):  
Daniel Ding ◽  
Jun Wang
Keyword(s):  
B Cell ◽  
Gene Rearrangement ◽  
B Cell Lymphoma ◽  
Plasmablastic Lymphoma ◽  
Proliferation Index ◽  
Chromosome 18 ◽  
Ki 67 ◽  
Hiv Test ◽  
Myc Gene ◽  
Bcl2 Gene

Abstract Objectives To describe a case of plasmablastic lymphoma with an atypical immunophenotype in an unusual location. Methods We received this specimen for routine processing after the patient underwent an incision and drainage surgery. The specimen was sent to hematopathology for workup of lymphoma. We then ordered several special stains and molecular tests to confirm our diagnosis of plasmablastic lymphoma. Results Histologic examination of the tissue reveals sheets of large plasmablastic cells with eccentrically located round nuclei, prominent nucleoli, and cartwheel chromatin. Frequent apoptotic bodies and mitotic figures are present. Immunohistochemistry shows strong, diffuse positivity for leukocyte common antigen CD45, CD10, plasmacytic markers (MUM1, CD79a) and negativity for B-cell markers (CD20, PAX5) and CD138. The Ki-67 proliferation index is very high (>90%) and Epstein-Barr virus encoded small RNA (EBER) in situ hybridization is diffusely positive. Molecular FISH testing for aggressive B-cell lymphoma genetic abnormalities is positive for MYC gene rearrangement in 68% of nuclei and the loss of one copy of the BCL2 gene on chromosome 18 in 64% of nuclei. Conclusion This case of plasmablastic lymphoma is unique due to its atypical presentation as a perianal mass in a young patient with undiagnosed HIV. Usually, this lymphoma presents as an oral lesion in an older patient with HIV. In addition, immunohistochemistry showed an unusual immunophenotype (CD138–, MUM1+, CD45+, CD10+). Most plasmablastic lymphomas are CD138+, MUM1+, and CD45– but this specimen is CD138–, MUM1+, and CD45+. Further testing shows EBER positivity, a positive HIV test, and MYC gene rearrangement, which confirms our diagnosis of a plasmablastic lymphoma. Of note, genetic analysis also reveals a loss of one copy of the BCL2 gene on chromosome 18 in 64% of nuclei.

scholarly journals Nfatc1 and Tumor Associated Macrophages Affect Progression of Certain Subsets of Diffuse Large B Cell Lymphoma Non-Gcb Subtypes

2021 ◽  
Vol 5 (2) ◽  
pp. 345-353
Author(s):  
Krisna Murti ◽  
Muslina Muslina ◽  
Ika Kartika ◽  
Rachmat Hidayat ◽  
Ella Amalia
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Therapeutic Strategies ◽  
Proliferation Index ◽  
Ki 67 ◽  
Large B Cell Lymphoma ◽  
Positive Expression ◽  
Activated T Cell ◽  
Large B Cell

A B S T R A C TIntroduction Diffuse large B cell lymphoma (DLBCL) is the most common type ofnon-Hodgkin lymphoma among B cell lymphomas. The interaction of tumor cellswith their microenvironment (tumor microenvironment, TME) leads to progressivityof malignancy. CD163 + macrophages known as components of TME. Nuclear factorof activated T cell (NFATc1) and MYC are important transcription factors inmalignant transformation and progression. Therapeutic strategies were fastdeveloped, nevertheless, efforts to decrease DLBCL morbidity and mortality areunsatisfied, therefore,new markers for prognosis and or therapeutic options of thepatients are necessary. This study was aimed to investigate NFATc1 expression inDLBCL and its TME. Methods: Thirty-two paraffin blocks were selected thenimmunostained for expression of NFATc1, MYC, and CD163. Clinopathologic datai.e. ages, gender, and proliferation index Ki-67 were obtained. Data was analyzedby statistics Result: Positive expression of CD163 and NFATc1 was among 55%and 45% of cases respectively. All DLBCL cases in this study were non-GCBsubtype and more patients were under 60 years (66%). Positive expression ofCD163 was higher in males (69%) and in patients under 60 years (63%). Tissuespositive for both NFATc1 and CD163 was observed higher among males andpatients under 60 years. Conclusion: NFATc1 may affect development and orprogression of certain subsets of DLBCL non-GCB subtype.

scholarly journals “Double hit” follicular lymphoma with low proliferation index: A unique case and literature review

2017 ◽  
Vol 3 (2) ◽  
Author(s):  
Pardis Vafaii ◽  
Haipeng Shao
Keyword(s):  
Follicular Lymphoma ◽  
B Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Proliferation Index ◽  
Low Grade ◽  
Gene Rearrangements ◽  
Double Hit ◽  
Indolent Disease

<p>“Double hit” lymphomas (DHLs) are aggressive B-cell lymphomas with concurrent <em>c-MYC</em> and <em>BCL2</em> and/or <em>BCL6</em> gene rearrangements. DHLs are usually classified morphologically as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, and less commonly as DLBCL. Follicular lymphoma (FL) is characterized genetically by the presence of <em>IGH-BCL2</em> rearrangement. A subset of DHLs arises from FL by the acquisition of <em>c-MYC</em> gene rearrangement during disease progression, but FL with concurrent <em>IGH-BCL2</em> and <em>c-MYC</em> gene initial rearrangements is rarely reported. The few reported cases had different clinical courses, including some with indolent disease. We report a case of “double hit” low grade FL with both <em>c-MYC</em> and <em>BCL2</em> gene rearrangements but at low proliferation rate. Unlike the usual DHLs with aggressive clinical course, our patient showed at least partial response to intense chemotherapy. Review of the literature shows a few similar cases with variable clinical course, including a few indolent cases. These patients appear to respond better with more intense chemotherapy for DHL.</p>

scholarly journals Case Report: Multiple Chromosomal Translocations Including Novel CIITA-CREBBP Fusion and Mutations in a Follicular Lymphoma

2021 ◽  
Vol 11 ◽  
Author(s):  
Huan-You Wang ◽  
Ethan S. Sokol ◽  
Aaron M. Goodman ◽  
Andrew L. Feldman ◽  
Carolyn M. Mulroney
Keyword(s):  
Follicular Lymphoma ◽  
Mhc Class Ii ◽  
Fusion Gene ◽  
B Cell Lymphoma ◽  
Class Ii ◽  
Chromosomal Translocations ◽  
Proliferation Index ◽  
Low Grade ◽  
Creb Binding Protein ◽  
Ki 67

The pathogenesis of follicular lymphoma is a multi-step process, in which chromosomal translocation between immunoglobulin heavy chain (IgH) and anti-apoptotic B-cell lymphoma 2 (BCL2), namely IgH-BCL2, is an earliest step, followed by other genetic/genomic alterations including but not limited to mutation of CREB binding protein (CREBBP). MHC class II transactivator (CIITA) is a transcription regulator responsible for expression of MHC class II molecules including HLA-DR in human. We report herein a novel fusion gene involving CIITA and CREBBP in a patient with a low-grade follicular lymphoma (FL) but with high Ki-67 proliferation index. In addition, our patient also harbors CREBBP mutation. Together, we postulate that total loss of CREBBP function may contribute, in part, to the lymphoma genesis. Furthermore, this patient has addition rare (TBL1XR1-TP63) and common (IgH-BCL2) chromosomal translocations and multiple mutations including BCL2, BRAF, MUTYH, and STAT6.

Expression of Skp2, a p27Kip1 ubiquitin ligase, in malignant lymphoma: correlation with p27Kip1 and proliferation index

Blood ◽  
2002 ◽  
Vol 100 (8) ◽  
pp. 2950-2956 ◽  
Author(s):  
Megan S. Lim ◽  
Ann Adamson ◽  
Zhaosheng Lin ◽  
Bayardo Perez-Ordonez ◽  
Richard C. K. Jordan ◽  
...  
Keyword(s):  
B Cell ◽  
Ubiquitin Ligase ◽  
Peripheral Blood Lymphocytes ◽  
Proliferation Index ◽  
Low Grade ◽  
B Cell Lymphomas ◽  
Ki 67 ◽  
Skp2 Expression ◽  
Aggressive Lymphomas ◽  
F Box Protein

Reduced levels of p27Kip1 are frequent in human cancers and have been associated with poor prognosis. Skp2, a component of the Skp1-Cul1-F-box protein (SCF) ubiquitin ligase complex, has been implicated in p27Kip1 degradation. Increased Skp2 levels are seen in some solid tumors and are associated with reduced p27Kip1. We examined the expression of these proteins using single and double immunolabeling in a large series of lymphomas to determine if alterations in their relative levels are associated with changes in cell proliferation and lymphoma subgroups. We studied the expression of Skp2 in low-grade and aggressive B-cell lymphomas (n = 86) and compared them with p27Kip1 and the proliferation index (PI). Fifteen hematopoietic cell lines and peripheral blood lymphocytes were studied by Western blot analysis. In reactive tonsils, Skp2 expression was limited to proliferating germinal center and interfollicular cells. Skp2 expression in small lymphocytic lymphomas (SLLs) and follicular lymphomas (FCLs) was low (mean percentage of positive tumor cells, less than 20%) and was inversely correlated (r = −0.67;P < .0001) with p27Kip1 and positively correlated with the PI (r = 0.82;P < .005). By contrast, whereas most mantle cell lymphomas (MCLs) demonstrated low expression of p27Kip1 and Skp2, a subset (n = 6) expressed high Skp2 (exceeding 20%) with a high PI (exceeding 50%). Skp2 expression was highest in diffuse large B-cell lymphomas (DLBCLs) (mean, 22%) and correlated with Ki-67 (r = 0.55;P < .005) but not with p27Kip1. Cytoplasmic Skp2 was seen in a subset of aggressive lymphomas. Our data provide evidence for p27Kip1 degradative function of Skp2 in low-grade lymphomas. The absence of this relationship in aggressive lymphomas suggests that other factors contribute to deregulation of p27Kip1 expression in these tumors.

scholarly journals Immunophenotypic Analysis of AIDS-Related Diffuse Large B-Cell Lymphoma and Clinical Implications in Patients From AIDS Malignancies Consortium Clinical Trials 010 and 034

2009 ◽  
Vol 27 (30) ◽  
pp. 5039-5048 ◽  
Author(s):  
Amy Chadburn ◽  
April Chiu ◽  
Jeannette Y. Lee ◽  
Xia Chen ◽  
Elizabeth Hyjek ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Hiv Infection ◽  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Response To Therapy ◽  
Proliferation Index ◽  
Ki 67 ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Purpose Diffuse large B-cell lymphoma (DLBCL) represents a clinically heterogeneous disease. Models based on immunohistochemistry predict clinical outcome. These include subdivision into germinal center (GC) versus non-GC subtypes; proliferation index (measured by expression of Ki-67), and expression of BCL-2, FOXP1, or B-lymphocyte-induced maturation protein (Blimp-1)/PRDM1. We sought to determine whether immunohistochemical analyses of biopsies from patients with DLBCL having HIV infection are similarly relevant for prognosis. Patients and Methods We examined 81 DLBCLs from patients with AIDS in AMC010 (cyclophosphamide, doxorubicin, vincristine, and prednisone [CHOP] v CHOP-rituximab) and AMC034 (etoposide, doxorubicin, vincristine, prednisone, and dose-adjusted cyclophosphamide plus rituximab concurrent v sequential) clinical trials and compared the immunophenotype with survival data, Epstein-Barr virus (EBV) positivity, and CD4 counts. Results The GC and non-GC subtypes of DLBCL did not differ significantly with respect to overall survival or CD4 count at cancer presentation. EBV could be found in both subtypes of DLBCL, although less frequently in the GC subtype, and did not affect survival. Expression of FOXP1, Blimp-1/PRDM1, or BCL-2 was not correlated with the outcome in patients with AIDS-related DLBCL. Conclusion These data indicate that with current treatment strategies for lymphoma and control of HIV infection, commonly used immunohistochemical markers may not be clinically relevant in HIV-infected patients with DLBCL. The only predictive immunohistochemical marker was found to be Ki-67, where a higher proliferation index was associated with better survival, suggesting a better response to therapy in patients whose tumors had higher proliferation rates.

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