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. 2022 May 24;6(10):3189-3193.
doi: 10.1182/bloodadvances.2021006018.

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

Ekin Güney  1 , Calixto-Hope G Lucas  1 , Zhongxia Qi  2 , Jingwei Yu  2 , Ruth Zhang  1 , Robert S Ohgami  1   3 , James L Rubenstein  4 , Daniel R Boué  5 , Kristian Schafernak  6 , Gerald B Wertheim  7 , Sonika Dahiya  8 , Lisa Giulino-Roth  9   10 , Andishe Attarbaschi  11 , Matthew J Barth  12 , Shalin Kothari  13 , Oussama Abla  14 , Adam L Cohen  15   16   17 , Joe S Mendez  18 , Andrew Bollen  1 , Arie Perry  1 , Tarik Tihan  1 , Melike Pekmezci  1   3 , David A Solomon  1   3 , Kwun Wah Wen  1   3
Affiliations

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

Ekin Güney et al. Blood Adv. .
No abstract available

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Figures

Figure 1.
Figure 1.
Genomic analysis identifies a unique pediatric subtype of EBV-negative primary CNS large B-cell lymphoma. Clinical, histologic, and genetic features of the 12 pediatric and young-adult patients with PCNS-LBCL, alongside 6 conventional cases of PCNS-LBCL occurring in older adults. Categorization into pediatric- and adult-type tumor groups was performed according to recurrent molecular alterations involving MYD88, PRDM1, CDKN2A/B, HLA gene cluster, NFKBIE, GNA13, and TP53.
Figure 2.
Figure 2.
Pediatric MYD88-wildtype PCNS-LBCL is associated with favorable clinical outcomes compared with its adult-type counterpart. (A-F) Representative radiologic and histopathologic findings in pediatric- and adult-type PCNS-LBCL. Preoperative T1-weighted postcontrast magnetic resonance images of patients with pediatric type, MYD88-wildtype (A) or adult type, MYD88-mutant (D) PCNS-LBCL, showing overlapping radiologic features, including intraparenchymal mass lesions with diffuse enhancement and significant edema in the adjacent white matter. Histologic analysis showed sheets of malignant large B-cells diffusely expressing CD20. Hematoxylin and eosin, magnification ×400 (B,E); and CD20, original magnification, ×400 (C,F). (G) Kaplan-Meier survival plot of 7 patients with pediatric type, MYD88-wildtype PCNS-LBCL. (H) Kaplan-Meier meta-analysis of 53 pediatric and young-adult patients with PCNS-LBCL stratified by age ≤ 25 years vs 26 to 40 years.
See this image and copyright information in PMC

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