GCB-type is a favorable prognostic factor in primary CNS diffuse large B-cell lymphomas

Marcus, Chelsea; Maragkos, Georgios A; Alterman, Ron L.; Uhlmann, Erik J.; Pihán, Germán; Varma, Hemant

doi:10.1016/j.jocn.2020.11.031

Cited by 10 publications

(7 citation statements)

References 43 publications

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“…Four tumors in this group exhibited a small number of chromosomal gains/losses (<5/tumor), while the quantity of chromosomal aberrations was high (5+) in the remaining 4 tumors. No differences in radiologic or histologic features between these pediatric type PCNS-LBCL and their adult type counterparts was appreciable (Figure 2A-F Kaplan-Meier survival analysis demonstrated a 5-year overall survival (OS) of 100% for the pediatric type, MYD88-wildtype group, which was substantially longer than PCNS-LBCL in adult patients from previous studies [15][16][17][18][19][20] (Figure 2G, Sup. Tables 8-9).…”

Section: Clinical Trial Registration Information (If Any)mentioning

confidence: 79%

“…A Kaplan-Meier survival analysis demonstrated a 5-year overall survival (OS) of 100% for the pediatric-type, MYD88 wild-type group, which was substantially longer than PCNS-LBCL in adult patients in previous studies 15-20 ( Figure 2G ; supplemental Tables 8-9). We next performed meta-analysis of clinical outcome data, including 37 additional patients with PCNS-LBCL aged <40 years from 5 published studies, 2 , 21-24 along with 5 additional previously unpublished cases.…”

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confidence: 82%

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A genetically distinct pediatric subtype of primary CNS large B-cell lymphoma is associated with favorable clinical outcome

Güney¹,

Lucas²,

Qi³

et al. 2022

Blood Advances

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Primary central nervous system large B-cell lymphoma (PCNS-LBCL) occurs typically in older adults and only rarely in the pediatric population. The genomic landscape of PCNS-LBCL in children and young adults (YA) is not well-characterized. In this multi-institutional study, targeted next-generation DNA sequencing and chromosomal copy number analysis was performed on a cohort of 12 pediatric and YA (age<40 years) PCNS-LBCL patients without known immunodeficiency and correlated with clinicopathologic data. Based on genomic features, we identified two subgroups: a unique "Pediatric type, MYD88-wildtype" group (median age 14 years, range 7-25 years) was characterized by absence of MYD88 mutations but frequent genetic alterations in TP53 (6/8, 75%), NFKBIE (4/8, 50%), and GNA13 (4/8, 50%); and an "adult type, MYD88-mutant" group (median age 35 years, range 25-38 years) was defined by MYD88 hotspot mutations (4/4, 100%), with frequent PRDM1 mutation/deletion (3/4, 75%), CDKN2A/B homozygous deletion (3/4, 75%), and deletions of the HLA gene cluster (2/4, 50%). Kaplan-Meier analysis demonstrated that patients with pediatric type, MYD88-wildtype PCNS-LBCL had favorable outcomes (median survival: >100 months; 5-year-overall survival: 100%). In conclusion, we have identified a new pediatric type of PCNS-LBCL that is molecularly distinct from PCNS-LBCL occurring in adults, based on an absence of MYD88 mutation, CDKN2A/B homozygous deletion, deletion of HLA gene cluster, and paucity of CD79B and PRDM1 mutations, along with an enrichment for TP53, NFKBIE, and GNA13 mutations. Patients with pediatric type, MYD88-wildtype PCNS-LBCL often have long-term survival compared to their adult counterparts.

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Section: Clinical Trial Registration Information (If Any)mentioning

confidence: 79%

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confidence: 82%

A genetically distinct pediatric subtype of primary CNS large B-cell lymphoma is associated with favorable clinical outcome

Güney¹,

Lucas²,

Qi³

et al. 2022

Blood Advances

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“…The GCB types includes CD10(−), BCL6( + ), MUM1(−) immunotypes, and CD10( + ) immunotypes, with varying proportions 11,21 and generally a better prognosis. 22 This study classified DLBCL into GCB or non-GCB according to the Hans algorithm, and showed their association with clinicopathological parameters in patients. Indeed, there were 2 cases (Cases 2 and 4) consistent with the GCB type in this study: one case of HT from FL (Case 4), in which CD10 percentages in FCM were high, and the other case (Case 2) showing immunohistochemical CD10 positivity in the tumor tissue, where CD10 in FCM was relatively low, although there seems to be some discrepancy of results between FCM and immunohistochemistry.…”

Section: Discussionmentioning

confidence: 98%

Diffuse large b-cell lymphoma of the ocular adnexa with flow cytometry analysis and review of the literature

Mitamura

Kase

Suzuki

et al. 2022

European Journal of Ophthalmology

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Purpose The aim of this study was to evaluate the clinicopathological features and flow cytometry (FCM) of tumor tissues in ocular adnexal diffuse large B-cell lymphoma (DLBCL). Methods This retrospective, multicenter case study was designed to evaluate the clinical and immunohistochemical features of tumors. DLBCL was diagnosed based on histopathology, immunoglobulin (Ig) heavy chain gene rearrangement, and FCM in all surgically removed periocular tumor tissues. This study involved assessing percentages (%) of B-cell/T-cell markers, a natural killer cell marker, and cell-surface Ig kappa/lambda (κ/λ) expression measured by FCM analysis in tumor tissues. Results Eleven DLBCL patients (4 men and 7 women) with 11 tumors were enrolled in this study. The median age at the time of initial presentation was 73 years. The tumor cells were immunohistochemically positive for cluster of differentiation (CD) 20, while CD5 was negative in all 8 cases tested. At the time of ophthalmic diagnosis, two cases already showed systemic dissemination of DLBCL throughout the body. FCM of tumor tissues detected a high percentage of B-cell markers including CD19 and CD20 in all 11 tumors. One case with high CD10 levels in FCM was histologic transformation from follicular lymphoma. One case with a relatively low CD20 population involved a history of systemic treatments including intravenous rituximab. Conclusion Although caution should be exercised when interpreting the data, FCM is useful for not only supportive diagnosis complementary to immunohistochemistry, but also facilitates a better understanding of immunopathology including histologic transformation of follicular lymphoma to DLBCL in the ocular adnexa.

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“…Primary central nervous system DLBCL is a highly malignant brain disease and the germinal center subtype is rare [ 4 ]. Although this subtype is considered rare, the literature reports better clinical outcomes compared with non-germinal center subtype [ 5 ]. The disease in immunocompetent patients typically presents as a solitary lesion with predilection for the periventricular white matter, with possible involvement of the gray matter [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

Rodrigues,

Santos,

Martins

2024

Am J Case Rep

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Patient: Male, 44-year-old Final Diagnosis: Primary central nervous system diffuse large B-cell lymphoma Symptoms: Bilateral frontal headache Clinical Procedure: — Specialty: General and Internal Medicine • Neurosurgery • Radiology Objective: Unusual clinical course Background: Primary central nervous system diffuse large B-cell lymphoma (DLBCL) is an extremely aggressive brain disease that rarely affects immunocompetent non-elderly patients, particularly with hemorrhagic presentation. Brain magnetic resonance imaging (MRI) plays an important role in the diagnosis of this entity, which typically demonstrates restricted diffusion and a T2 hypointense appearance, suggesting hypercellularity. Case Report: A 44-year-old man came to the emergency department with a persistent and treatment-resistant bilateral frontal headache that had been bothering him for the past 3 weeks. Upon conducting a neurological assessment, the patient displayed temporal disorientation and incoherent speech, but without any observable motor deficits. A non-contrast enhanced brain computed tomography scan was carried out, revealing a hyperattenuating, space-occupying lesion and hemorrhage in the left hemisphere of the brain. Subsequently, brain MRI demonstrated hypointense signal on T2-weighted images, restricted diffusion, and homogeneous lesional contrast enhancement, suggesting a very cellular expansive lesion with hemorrhage. To establish a definitive diagnosis, a brain biopsy was undertaken, confirming the presence of DLBCL of the primary central nervous system (germinal center phenotype). Conclusions: Hemorrhagic presentation of primary central nervous system DLBCL occurs very rarely, particularly in non-elderly immunocompetent patients. Brain MRI plays an important role in the diagnosis of this entity, which allows differentiation from high-grade glial or other lesions that present more frequently with hemorrhage. Therefore, it is crucial to suspect lymphoma before surgical intervention for appropriate patient management.

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GCB-type is a favorable prognostic factor in primary CNS diffuse large B-cell lymphomas

Cited by 10 publications

References 43 publications

A genetically distinct pediatric subtype of primary CNS large B-cell lymphoma is associated with favorable clinical outcome

A genetically distinct pediatric subtype of primary CNS large B-cell lymphoma is associated with favorable clinical outcome

Diffuse large b-cell lymphoma of the ocular adnexa with flow cytometry analysis and review of the literature

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

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