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Review
. 2020 Aug 20:13:8323-8335.
doi: 10.2147/OTT.S192379. eCollection 2020.

Systemic Approach to Recurrent Primary CNS Lymphoma: Perspective on Current and Emerging Treatment Strategies

Matthias Holdhoff  1 , Nina Wagner-Johnston  1 , Mark Roschewski  2
Affiliations
Review

Systemic Approach to Recurrent Primary CNS Lymphoma: Perspective on Current and Emerging Treatment Strategies

Matthias Holdhoff et al. Onco Targets Ther. .

Abstract

There is no uniform standard of care for the treatment of refractory or recurrent primary central nervous lymphoma (r/r PCNSL). Many different systemic treatment regimens have been studied, but available data are based on small prospective or retrospective reports. There have been no randomized controlled trials in r/r PCNSL to date. Here, we provide an overview of published systemic regimens for the treatment of r/r PCNSL, as well as therapies that are under investigation. In addition, based on available data, we propose strategies of how to approach choice of therapy for different groups of patients in this disease setting. Patients can be mainly divided into three groups: 1) patients suitable for a re-challenge with high-dose methotrexate (HD-MTX)-based regimens and that may or may not be candidates for consolidation with high-dose chemotherapy with autologous stem cell transplant, 2) patients refractory to HD-MTX or that had early relapse, but suitable for an aggressive treatment strategy with re-induction with non-MTX-based therapy, possibly followed by high-dose chemotherapy with autologous transplant, and 3) patients not suitable for re-treatment with HD-MTX and that are not candidates for aggressive therapy. As PCNSL is a rare disease and as there is urgent need for better outcomes in r/r PCNSL, clinical trial participation is encouraged, especially in elderly or frail patients who are not candidates for high-dose chemotherapy and transplant.

Keywords: B-cell lymphoma; PCNSL; autologous stem cell transplant; ibrutinib; primary central nervous system lymphoma; recurrent PCNSL.

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Conflict of interest statement

Dr Matthias Holdhoff reports grant support from the National Cancer Institute (NCI), during the conduct of the study; advisory board or consultative work for Celgene, AbbVie, Inc., NewLink Genetics, BTG International Ltd., and DP Clinical, Inc., outside the submitted work. Dr Nina Wagner-Johnston is on the advisory board for ADC Therapeutics, CALIB-R, Verastem, Bayer, Gilead, and JUNO, outside the submitted work. The authors report no other conflicts of interest in this work.

References

    1. Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS. CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the united states in 2011–2015. Neuro Oncol. 2018;20(suppl_4):iv1iv86. doi: 10.1093/neuonc/noy131 - DOI - PMC - PubMed
    1. Grommes C, DeAngelis LM. Primary CNS lymphoma. J Clin Oncol. 2017;35(21):2410–2418. doi: 10.1200/JCO.2017.72.7602 - DOI - PMC - PubMed
    1. Hoffman S, Propp JM, McCarthy BJ. Temporal trends in incidence of primary brain tumors in the united states, 19851999. Neuro Oncol. 2006;8(1):27–37. doi: 10.1215/S1522851705000323 - DOI - PMC - PubMed
    1. Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer. 2011;105(9):1414–1418. doi: 10.1038/bjc.2011.357 - DOI - PMC - PubMed
    1. Shiels MS, Pfeiffer RM, Besson C, et al. Trends in primary central nervous system lymphoma incidence and survival in the U. S Br J Haematol. 2016;174(3):417–424. doi: 10.1111/bjh.14073 - DOI - PMC - PubMed