Advances in Primary Central Nervous System Lymphoma

Abstract

Purpose of review: Optimal initial management can have a significant impact in long-term outcome in primary CNS lymphoma. This article reviews recent advances and the state of the field.

Recent findings: Genomic analysis of CSF cell-free DNA has emerged as a new diagnostic tool for PCNSL. Treatment options have likewise evolved, with mature data from first-line chemotherapy-based prospective trials disclosing excellent results in younger (< 60-65) patients, with a cure achieved in a majority. However, results in older patients remain dismal, with several new salvage options under investigation including BTK pathway-targeted therapies, and CAR-T cell treatments. Meanwhile, low-dose radiation has emerged as an additional alternative for consolidation therapy. For younger PCNSL patients, the goal of treatment is now a cure, with the next frontier being the development of therapies affording optimized neurocognitive outcome and lower toxicity. Treatment for older patients remains however an unmet need, with several promising clinical trials ongoing.

Keywords: BTK-Bruton tyrosine kinase; DLBCL – diffuse large B cell lymphoma; HD-MTX – high-dose methotrexate; M-RT – methotrexate-rituximab-temozolomide; Primary central nervous system lymphoma – PCNSL; R-MVP – rituximab-methotrexate-vincristine-procarbazine.