Genomic Profiling Reveals Differences in Primary Central Nervous System Lymphoma and Large B-Cell Lymphoma, With Subtyping Suggesting Sensitivity to BTK Inhibition.


Journal

The oncologist
ISSN: 1549-490X
Titre abrégé: Oncologist
Pays: England
ID NLM: 9607837

Informations de publication

Date de publication:
18 01 2023
Historique:
received: 29 09 2021
accepted: 19 07 2022
pubmed: 8 11 2022
medline: 21 1 2023
entrez: 7 11 2022
Statut: ppublish

Résumé

B-cell primary central nervous system (CNS) lymphoma (PCL) is diffuse large B-cell lymphoma (DLBCL) confined to the CNS. Less than 50% of patients with PCL achieve complete remission with current therapies. We describe the findings from comprehensive genomic profiling (CGP) of a cohort of 69 patients with PCL, 36 cases of secondary CNS lymphoma (SCL), and 969 cases of DLBCL to highlight their differences and characterize the PCL cohort. In addition, we highlight the differences in frequency of germinal center B-cell like (GCB) and non-GCB subtypes and molecular subtypes, particularly MCD and EZH subtypes, between PCL and DLBCL. Sixty-nine cases of B-cell PCL, 36 cases of secondary CNS lymphoma (SCL), and 969 cases of DLBCL were evaluated by CGP of 405 genes via DNAseq and 265 genes via RNAseq for fusions (FoundationOne Heme). Tumor mutational burden (TMB) was calculated from 1.23 Mb of sequenced DNA. Genomic alterations with significant differences between PCL and DLBCL included MYD88, ETV6, PIM1, PRDM1, CXCR4, TP53, and CREBBP, while only MYD88 was significantly different between SCL and DLBCL. PCL cases were significantly enriched for the MCD molecular subtypes, which have an excellent response to BTKi. We report a patient with a durable complete response to BTKi consistent with their genomic profile. EBV status, CD274 amplification, and TMB status suggest that 38% of PCL patients may benefit from ICPI; however further study is warranted. CGP of PCLs reveals biomarkers, genomic alterations, and molecular classifications predictive of BTKi efficacy and potential ICPI efficacy. Given the limitations of standard of care for PCL, CGP is critical to identify potential therapeutic approaches for patients in this rare form of lymphoma.

Sections du résumé

BACKGROUND
B-cell primary central nervous system (CNS) lymphoma (PCL) is diffuse large B-cell lymphoma (DLBCL) confined to the CNS. Less than 50% of patients with PCL achieve complete remission with current therapies. We describe the findings from comprehensive genomic profiling (CGP) of a cohort of 69 patients with PCL, 36 cases of secondary CNS lymphoma (SCL), and 969 cases of DLBCL to highlight their differences and characterize the PCL cohort. In addition, we highlight the differences in frequency of germinal center B-cell like (GCB) and non-GCB subtypes and molecular subtypes, particularly MCD and EZH subtypes, between PCL and DLBCL.
MATERIALS AND METHODS
Sixty-nine cases of B-cell PCL, 36 cases of secondary CNS lymphoma (SCL), and 969 cases of DLBCL were evaluated by CGP of 405 genes via DNAseq and 265 genes via RNAseq for fusions (FoundationOne Heme). Tumor mutational burden (TMB) was calculated from 1.23 Mb of sequenced DNA.
RESULTS
Genomic alterations with significant differences between PCL and DLBCL included MYD88, ETV6, PIM1, PRDM1, CXCR4, TP53, and CREBBP, while only MYD88 was significantly different between SCL and DLBCL. PCL cases were significantly enriched for the MCD molecular subtypes, which have an excellent response to BTKi. We report a patient with a durable complete response to BTKi consistent with their genomic profile. EBV status, CD274 amplification, and TMB status suggest that 38% of PCL patients may benefit from ICPI; however further study is warranted.
CONCLUSION
CGP of PCLs reveals biomarkers, genomic alterations, and molecular classifications predictive of BTKi efficacy and potential ICPI efficacy. Given the limitations of standard of care for PCL, CGP is critical to identify potential therapeutic approaches for patients in this rare form of lymphoma.

Identifiants

pubmed: 36342081
pii: 6808327
doi: 10.1093/oncolo/oyac190
pmc: PMC9847534
doi:

Substances chimiques

Myeloid Differentiation Factor 88 0
Biomarkers, Tumor 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

e26-e35

Informations de copyright

© The Author(s) 2022. Published by Oxford University Press.

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Auteurs

Eric A Severson (EA)

Foundation Medicine, Morrisville, NC, USA.

James Haberberger (J)

Foundation Medicine, Morrisville, NC, USA.

Amanda Hemmerich (A)

Foundation Medicine, Morrisville, NC, USA.

Richard S P Huang (RSP)

Foundation Medicine, Morrisville, NC, USA.

Claire Edgerly (C)

Foundation Medicine, Morrisville, NC, USA.

Kelsie Schiavone (K)

Foundation Medicine, Morrisville, NC, USA.

Adib Najafian (A)

Foundation Medicine, Morrisville, NC, USA.

Matthew Hiemenz (M)

Foundation Medicine, Cambridge, MA, USA.

Mirna Lechpammer (M)

Foundation Medicine, Cambridge, MA, USA.

Jo-Anne Vergilio (JA)

Foundation Medicine, Cambridge, MA, USA.

Glenn Lesser (G)

Wake Forest Baptist Comprehensive Cancer Center, Wake Forest School of Medicine, Winston-Salem, NC, USA.

Roy Strowd (R)

Wake Forest Baptist Comprehensive Cancer Center, Wake Forest School of Medicine, Winston-Salem, NC, USA.

Julia Elvin (J)

Foundation Medicine, Cambridge, MA, USA.

Jeffrey S Ross (JS)

Foundation Medicine, Cambridge, MA, USA.

Priti Hegde (P)

Foundation Medicine, Cambridge, MA, USA.

Brian Alexander (B)

Foundation Medicine, Cambridge, MA, USA.

Samuel Singer (S)

Hackensack University Medical Center, Hackensack, NJ, USA.

Shakti Ramkissoon (S)

Foundation Medicine, Morrisville, NC, USA.
Wake Forest Baptist Comprehensive Cancer Center, Wake Forest School of Medicine, Winston-Salem, NC, USA.

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