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Original Articles

EBV-positive PCNSL in older patients: incidence, characteristics, tumor pathology, and outcomes across a large multicenter cohort

Prashasti Agrawala Weill Cornell Medicine/New York Presbyterian Hospital, New York, NY, USA

Kevin A. Davidb Rutgers Cancer Institute of New Jersey, New Brunswick, NJ, USA

Zhengming Chena Weill Cornell Medicine/New York Presbyterian Hospital, New York, NY, USA

https://orcid.org/0000-0001-9437-6452

Suchitra Sundaramc Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA

Seo-Hyun Kimd Department of Internal Medicine, Division of Hematology, Oncology and Cell Therapy, Rush University Medical Center, Chicago, IL, USA

Ryan Vacae Department of Medicine, Penn State University College of Medicine, Hershey, PA, USA

Yong Linb Rutgers Cancer Institute of New Jersey, New Brunswick, NJ, USA

Samuel Singerf John Theurer Cancer Center, Hackensack, NJ, USA

Mary-Kate Malecekg Department of Medicine, Division of Hematology & Oncology, Washington University School of Medicine, University City, MO, USA

Jordan Carterb Rutgers Cancer Institute of New Jersey, New Brunswick, NJ, USA

Adam Zayach Department of Medicine, Alpert Medical School of Brown University, Providence, RI, USA

https://orcid.org/0000-0002-7591-2069

Myung Sun Kimi Department of Internal Medicine, Oregon Health and Science University, Portland, OR, USA

Nishitha Reddyj Department of Medicine, Division of Hematology & Oncology, Vanderbilt University Medical Center, Nashville, TN, USA

Douglas Neyk Department of Neurology, University of Colorado, Aurora, CO, USA

Alma Habibl Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota School of Medicine, Minneapolis, MN, USA

Christopher Strousem Department of Internal Medicine, University of Iowa, Iowa City, IA, USA

Jerome Grabern Department of Neurology, University of Washington, Seattle, WA, USA

https://orcid.org/0000-0001-5357-2932

Veronika Bachanoval Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota School of Medicine, Minneapolis, MN, USA

Sidra Salmano Department of Medicine and Cancer Biology, Loyola University, Chicago, IL, USA

Jean A. Vendiolao Department of Medicine and Cancer Biology, Loyola University, Chicago, IL, USA

Nasheed Hossaino Department of Medicine and Cancer Biology, Loyola University, Chicago, IL, USA

Mazie Tsangp Department of Hematology/Oncology, University of California San Francisco, San Francisco, CA, USA

Ajay Majorq Department of Medicine, Section of Hematology/Oncology, University of Chicago, Chicago, IL, USA

https://orcid.org/0000-0001-7261-1335

Maher K. Gandhir Princess Alexandria Hospital, Brisbane, Australia

Colm Keaner Princess Alexandria Hospital, Brisbane, Australia

David A. Bonds Department of Medicine, Division of Hematology, The Ohio State University, Columbus, OH, USA

https://orcid.org/0000-0003-0773-1829

Matthew Folstadt Department of Medicine, Division of Hematology, Oncology and Palliative Care, University of Wisconsin, Madison, WI, USA

Julie Changt Department of Medicine, Division of Hematology, Oncology and Palliative Care, University of Wisconsin, Madison, WI, USA

Angel Mier-Hicksc Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA

Pallawi Torkac Roswell Park Comprehensive Cancer Center, Buffalo, NY, USA

Priya Rajakumard Department of Internal Medicine, Division of Hematology, Oncology and Cell Therapy, Rush University Medical Center, Chicago, IL, USA

Parameswaran Venugopald Department of Internal Medicine, Division of Hematology, Oncology and Cell Therapy, Rush University Medical Center, Chicago, IL, USA

Stephanie Bergo Department of Medicine and Cancer Biology, Loyola University, Chicago, IL, USA

Michael Glantze Department of Medicine, Penn State University College of Medicine, Hershey, PA, USA

Samuel A. Goldlustf John Theurer Cancer Center, Hackensack, NJ, USA

Rahul Matnaniu Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA

Pallavi Kumaru Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA

Thomas A. Ollilah Department of Medicine, Alpert Medical School of Brown University, Providence, RI, USA

https://orcid.org/0000-0003-0102-6491

Johnny Caii Department of Internal Medicine, Oregon Health and Science University, Portland, OR, USA

Stephen E. Spurgeoni Department of Internal Medicine, Oregon Health and Science University, Portland, OR, USA

Alex G. Siegm Department of Internal Medicine, University of Iowa, Iowa City, IA, USA

Joseph Clevelandp Department of Hematology/Oncology, University of California San Francisco, San Francisco, CA, USA

Narendranath Epperlas Department of Medicine, Division of Hematology, The Ohio State University, Columbus, OH, USA

Reem Karmaliv Division of Hematology Oncology, Northwestern University, Chicago, IL, USA

Seema Naike Department of Medicine, Penn State University College of Medicine, Hershey, PA, USA

Sonali M. Smithq Department of Medicine, Section of Hematology/Oncology, University of Chicago, Chicago, IL, USA

James L. Rubensteinp Department of Hematology/Oncology, University of California San Francisco, San Francisco, CA, USA

Brad S. Kahlg Department of Medicine, Division of Hematology & Oncology, Washington University School of Medicine, University City, MO, USA

Amy Chadburna Weill Cornell Medicine/New York Presbyterian Hospital, New York, NY, USA

Andrew M. Evensb Rutgers Cancer Institute of New Jersey, New Brunswick, NJ, USA

Peter Martina Weill Cornell Medicine/New York Presbyterian Hospital, New York, NY, USACorrespondence[email protected]

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Abstract

The objective of this multicenter retrospective study was to examine the incidence, patient characteristics, pathology, and outcomes associated with Epstein-Barr virus (EBV)-related CNS lymphoma (CNSL) in older patients. Among 309 CNSL patients aged ≥60, 11.7% had EBV + tumors of which 72.2% were solid organ transplant (SOT)-related post-transplant lymphoproliferative disorders (PTLD). Younger age, SOT or autoimmune disease, and immunosuppressive treatment correlated highly with EBV-positivity. EBV + tumors were associated with absent C-MYC and BCL6 expression. EBV + PTLD was more likely to be associated with the absence of CD5 expression. EBV + non-PTLD had better median OS (not reached) compared to EBV + PTLD (10.8 months) and EBV-negative patients (43 months). Multivariable Cox regression analysis showed that age, performance status, and PTLD were negative predictors of OS. EBV status and immunosuppressive treatment were not correlated with OS. Our findings merit further investigation of EBV + PCNSL tumors and EBV-directed therapies.

Keywords:

Acknowledgements

Peter Martin and Amy Chadburn were supported by the following grant: NIH R01 CA260691.

Authors contributions

All authors provided data and reviewed the manuscript. Andrew Evens, Kevin David, Peter Martin, and Prashasti Agrawal developed the study concept. Peter Martin, Zhengming Chen, and Prashasti Agrawal performed data analysis.

Disclosure statement

No potential conflict of interest was reported by the author(s).

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EBV-positive PCNSL in older patients: incidence, characteristics, tumor pathology, and outcomes across a large multicenter cohort

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EBV-positive PCNSL in older patients: incidence, characteristics, tumor pathology, and outcomes across a large multicenter cohort

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