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How we treat primary central nervous system lymphoma
T Calimeri1, S Steffanoni1, F Gagliardi2
1Lymphoma Unit, Department of Onco-Hematology, IRCCS San Raffaele Scientific Institute, Milan.
ESMO Open
|July 16, 2021
Summary
Primary central nervous system lymphoma (PCNSL) is a potentially curable cancer, especially in younger patients. Research focuses on effective, less neurotoxic treatments crossing the blood-brain barrier, improving survival outcomes.
Area of Science:
- Hematology
- Oncology
- Neurology
- Clinical Trials
Background:
- Primary diffuse large B-cell lymphoma of the central nervous system (PCNSL) is a distinct entity from systemic DLBCL.
- Anthracycline-based chemotherapy shows poor outcomes in PCNSL due to low drug penetration across the blood-brain barrier.
- International research has focused on alternative strategies with improved drug CNS bioavailability.
Purpose of the Study:
- To review and analyze current literature on PCNSL management.
- To provide evidence-based recommendations for PCNSL patient care.
- To highlight advancements and remaining challenges in PCNSL treatment and diagnosis.
Main Methods:
- Review of international randomized trials and available literature.
- Analysis of chemotherapy regimens including high-dose methotrexate (HD-MTX), alkylating agents, and rituximab.
- Evaluation of consolidative strategies like autologous stem cell transplantation and whole-brain irradiation.
Main Results:
- Effective induction regimens include HD-MTX with alkylating agents and rituximab (with or without cytarabine).
- The MATRix regimen (HD-MTX/cytarabine/thiotepa/rituximab) followed by consolidation significantly improves survival in younger patients (≤70 years).
- Ongoing trials explore novel treatments like non-myeloablative chemotherapy, oral maintenance, and targeted agents (ibrutinib, lenalidomide).
Conclusions:
- PCNSL is a potentially curable malignancy, particularly in younger individuals.
- International cooperation has led to significant treatment advancements and improved survival.
- Further research is needed for optimal treatment of elderly patients and management of intraocular/meningeal disease, alongside diagnostic tool development.
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