Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 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

PubMed Disclaimer

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

References

    1. Ostrom QT, Patil N, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2013-2017. Neuro Oncol. 2020;22(12 suppl 2):iv1-iv96. - PMC - PubMed
    1. Attarbaschi A, Abla O, Ronceray L, et al. . Primary central nervous system lymphoma: initial features, outcome, and late effects in 75 children and adolescents [published correction appears in Blood Adv. 2020;4(6):1012.]. Blood Adv. 2019;3(24):4291-4297. - PMC - PubMed
    1. Dandachi D, Ostrom QT, Chong I, et al. . Primary central nervous system lymphoma in patients with and without HIV infection: a multicenter study and comparison with U.S national data. Cancer Causes Control. 2019;30(5):477-488. - PubMed
    1. Fukumura K, Kawazu M, Kojima S, et al. . Genomic characterization of primary central nervous system lymphoma. Acta Neuropathol. 2016;131(6):865-875. - PubMed
    1. Mrugala MMRJ, Rubenstein JL, Ponzoni M, Batchelor TT. Insights into the biology of primary central nervous system lymphoma. Curr Oncol Rep. 2009;11(1):73-80. - PMC - PubMed

Publication types

MeSH terms