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Published on: March 30, 2018
Primary central nervous system lymphoma
Jennifer I Stern1, Jeffrey J Raizer
1Department of Neurology, Feinberg School of Medicine, Northwestern University, 710 North Lake Shore Dr., Chicago, IL 60611, USA.
Insights
Primary central nervous system lymphoma (PCNSL) is a rare cancer affecting the brain. While treatments improve survival, high rates of relapse and neurotoxicity necessitate exploring new therapeutic strategies.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Primary central nervous system lymphoma (PCNSL) is a non-Hodgkin's lymphoma exclusively affecting the central nervous system.
- It occurs in both immunocompetent and immunodeficient individuals, with Epstein-Barr virus association in the latter.
- PCNSL can involve the brain, leptomeninges, spinal cord, and eyes.
Purpose of the Study:
- To summarize current understanding and treatment approaches for primary central nervous system lymphoma.
- To highlight challenges in PCNSL management, including relapse rates and treatment-related toxicities.
- To discuss emerging therapeutic strategies and the ongoing search for optimal treatment regimens.
Main Methods:
- Review of existing literature on PCNSL diagnosis and treatment.
- Analysis of outcomes associated with methotrexate-based chemotherapy and radiation therapy.
- Examination of recent advancements including immunotherapy and stem-cell transplantation.
Main Results:
- High-dose methotrexate and whole-brain radiation have improved survival but cause significant neurotoxicity in older patients.
- Despite current treatments, approximately 50% of patients experience disease relapse.
- Novel approaches like rituximab, stem-cell transplantation, and radiation-sparing regimens are under investigation.
Conclusions:
- Current treatments for PCNSL offer survival benefits but are limited by relapse and neurotoxicity.
- The optimal treatment strategy for PCNSL remains undetermined, particularly in elderly patients.
- Ongoing research focuses on refining existing therapies and exploring novel agents to improve outcomes and reduce side effects.
Abstract:
Primary central nervous system lymphoma is a stage 1E non-Hodgkin's lymphoma confined to the nervous system. It is seen in immunocompetent and immunodeficient populations, the latter group associated with the Epstein-Barr virus. Primary central nervous system lymphoma can affect the brain, leptomeninges, spinal cord or eyes. The institution of high-dose methotrexate-based regimens and whole-brain radiation therapy has significantly increased survival, but neurotoxicity is high in patients over 60 years of age. Despite these advances, 50% of patients initially treated will relapse. Recent investigations include the use of rituximab (immunotherapy) and stem-cell transplantation, as well as regimens without whole-brain radiation therapy in the elderly. The optimal treatment regimen is yet to been determined.
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