TY - JOUR
T1 - Primary large B-cell lymphoma of the central nervous system
T2 - A reappraisal of CD5-positive cases based on clinical, pathological, and molecular evaluation
AU - Yamada, Seiji
AU - Satou, Akira
AU - Tsuyuki, Yuta
AU - Iba, Sachiko
AU - Okumura, Yuka
AU - Ishikawa, Eri
AU - Ito, Hideaki
AU - Kogure, Yasunori
AU - Goto, Naoe
AU - Tanikawa, Motoki
AU - Shimada, Kazuyuki
AU - Tsukamoto, Tetsuya
AU - Karube, Kennosuke
AU - Yokoo, Hideaki
AU - Kataoka, Keisuke
AU - Tomita, Akihiro
AU - Mase, Mitsuhito
AU - Nakamura, Shigeo
N1 - Publisher Copyright:
© 2024 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.
PY - 2025/1
Y1 - 2025/1
N2 - CD5 expression is seen in 5%–10% of de novo diffuse large B-cell lymphomas (DLBCLs). Primary large B-cell lymphoma of the central nervous system (PCNS-LBCL) also exhibits CD5 expression in a minority of cases, however, clinicopathological and molecular features remain largely unclarified. Here we present the clinical, molecular, and pathological features of 11 CD5-positive (+) PCNS-LBCL cases, occupying 6.7% of all 165 PCNS-LBCLs diagnosed in our institutions. While CD5+ systemic DLBCL has been recognized as a distinctive subgroup showing an aggressive clinical course, no obvious differences were found between CD5+ and CD5-negative subgroups among the present CNS patients clinically. MYD88 p.L265P and CD79B p.Y196 mutations were detected in eight (73%) and seven (64%) cases, respectively, supporting previous reports. Notably, the microenvironmental immune cells were universally PD-L1/CD274-positive, and the higher levels tended to present favorable overall survival, as already evidenced in the PCNS-LBCL series. In contrast, neoplastic PD-L1/CD274 expression was undetectable in all cases. Indeed, no structural variations or copy number alterations involving PD-1 ligands were detected by targeted-capture sequencing and fluorescence in situ hybridization. While further studies are warranted, we may have confirmed similarity between PCNS-LBCLs and intravascular large B-cell lymphomas from a molecular standpoint.
AB - CD5 expression is seen in 5%–10% of de novo diffuse large B-cell lymphomas (DLBCLs). Primary large B-cell lymphoma of the central nervous system (PCNS-LBCL) also exhibits CD5 expression in a minority of cases, however, clinicopathological and molecular features remain largely unclarified. Here we present the clinical, molecular, and pathological features of 11 CD5-positive (+) PCNS-LBCL cases, occupying 6.7% of all 165 PCNS-LBCLs diagnosed in our institutions. While CD5+ systemic DLBCL has been recognized as a distinctive subgroup showing an aggressive clinical course, no obvious differences were found between CD5+ and CD5-negative subgroups among the present CNS patients clinically. MYD88 p.L265P and CD79B p.Y196 mutations were detected in eight (73%) and seven (64%) cases, respectively, supporting previous reports. Notably, the microenvironmental immune cells were universally PD-L1/CD274-positive, and the higher levels tended to present favorable overall survival, as already evidenced in the PCNS-LBCL series. In contrast, neoplastic PD-L1/CD274 expression was undetectable in all cases. Indeed, no structural variations or copy number alterations involving PD-1 ligands were detected by targeted-capture sequencing and fluorescence in situ hybridization. While further studies are warranted, we may have confirmed similarity between PCNS-LBCLs and intravascular large B-cell lymphomas from a molecular standpoint.
KW - CD5
KW - IVLBCL
KW - MCD subtype
KW - PCNS-LBCL
KW - PD-L1
UR - http://www.scopus.com/inward/record.url?scp=85211444217&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85211444217&partnerID=8YFLogxK
U2 - 10.1111/pin.13496
DO - 10.1111/pin.13496
M3 - Article
C2 - 39660959
AN - SCOPUS:85211444217
SN - 1320-5463
VL - 75
SP - 11
EP - 20
JO - Pathology International
JF - Pathology International
IS - 1
ER -