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Patterns of relapse after successful completion of initial therapy in primary central nervous system lymphoma: a case series

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Abstract

Purpose

Primary central nervous system lymphoma (PCNSL) is a subtype of non-Hodgkin’s lymphoma that involves the brain, spinal cord, or leptomeninges, without evidence of systemic disease. This rare disease accounts for ~ 3% of all primary central nervous system (CNS) tumors. Methotrexate-based regimens are the standard of care for this disease with overall survival rates ranging from 14 to 55 months. Relapse after apparent complete remission can occur. We sought to understand the outcomes of patients who relapsed.

Methods

This is an IRB-approved investigation of patients treated at our institution between 12/31/2004 and 10/12/2016. We retrospectively identified all cases of PCNSL as part of a database registry and evaluated these cases for demographic information, absence or presence of relapse, location of relapse, treatment regimens, and median relapse-free survival.

Results

This analysis identified 44 patients with a pathologically confirmed diagnosis of PCNSL. Mean age at diagnosis was 63.1 years (range 20–86, SD = 13.2 years). Of the 44 patients, 28 patients successfully completed an initial treatment regimen without recurrence or toxicity that required a change in therapy. Relapse occurred in 11 patients with the location of relapse being in the CNS only (n = 5), vitreous fluid only (n = 1), outside CNS only (n = 3), or a combination of CNS and outside of the CNS (n = 2). Sites of relapse outside of the CNS included testes (n = 1), lung (n = 1), adrenal gland (n = 1), kidney/adrenal gland (n = 1), and retroperitoneum (n = 1). Median relapse-free survival after successful completion of therapy was 6.7 years (95% CI 1.1, 12.6).

Conclusion

After successful initial treatment, PCNSL has a propensity to relapse, and this relapse can occur both inside and outside of the CNS. Vigilant monitoring of off-treatment patients with a history of PCNSL is necessary to guide early diagnosis of relapse and to initiate aggressive treatment.

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Funding

There was no funding for this study.

Author information

Authors and Affiliations

  1. Department of Pharmacy, Duke University Medical Center, Durham, NC, USA

    Mallika P. Patel

  2. Department of Radiation Oncology, Duke University Medical Center, Durham, NC, USA

    John P. Kirkpatrick

  3. Department of Neurosurgery, Duke University Medical Center, Durham, NC, USA

    Margaret O. Johnson, Eric S. Lipp, Elizabeth S. Miller, Annick Desjardins, Dina Randazzo, Henry S. Friedman, David M. Ashley & Katherine B. Peters

  4. Department of Biostatistics and Bioinformatics, Duke University Medical Center, Durham, NC, USA

    James E. Herndon II

  5. Department of Neurology, Duke University Medical Center, Durham, NC, USA

    Annick Desjardins, Dina Randazzo & Katherine B. Peters

  6. Department of Pediatrics, Duke University Medical Center, Durham, NC, USA

    Henry S. Friedman & David M. Ashley

  7. Duke Cancer Institute, Duke University Medical Center, Durham, NC, USA

    Mallika P. Patel, John P. Kirkpatrick, Margaret O. Johnson, Patrick Healy, James E. Herndon II, Eric S. Lipp, Elizabeth S. Miller, Annick Desjardins, Dina Randazzo, Henry S. Friedman, David M. Ashley & Katherine B. Peters

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  1. Mallika P. Patel
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Corresponding author

Correspondence to Mallika P. Patel.

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Conflict of interest

John P. Kirkpatrick reports grants from Varian Medical Systems. He is also an owner in limited partnership for ClearSight RT Products, not related to this work. Annick Desjardins owns stock in Istari Oncology. She has a consulting/advisory role for the following companies: Celgene, Istari Oncology and Orbus Therapeutics. She has received research funding from the following companies: Orbus Therapeutics, Genentech/Roche, Symphogen, and Triphase Accelerator Corp. Dina Randazzo has a consulting/advisory role for the following company: Optune. She has receiving research funding from the following company: Medicenna. Henry S. Friedman owns stock and other ownership interests in 2× Oncology and Istari Oncology. He has received honoraria from and has a consulting/advisory role for Genentech/Roche. He is on the speaker’s bureau for Genentech/Roche and has received travel, accommodations and expenses. David M. Ashley owns stock in Diverse Biotechnology. He has a consulting/advisory role for the following companies: Istari Oncology, Inc and The Jackson Laboratory. He has received research funding from the following companies: Midatech Pharma Plc. Katherine B. Peters has a consulting/advisory role for the following companies: Novocure, Agios, Eisai, Abbvie, Boehringer Ingelheim, and Monteris Medical. She has received research funding from the following companies: Agios, Abbvie, Bristol-Myers Squibb, Monteris Medical, and BioMimetix. Mallika P. Patel, Margaret O. Johnson, Patrick Healy, James E. Herndon II, Eric S. Lipp and Elizabeth S. Miller declared that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Patel, M.P., Kirkpatrick, J.P., Johnson, M.O. et al. Patterns of relapse after successful completion of initial therapy in primary central nervous system lymphoma: a case series. J Neurooncol 147, 477–483 (2020). https://doi.org/10.1007/s11060-020-03446-3

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  • DOI: https://doi.org/10.1007/s11060-020-03446-3

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