The Challenge of Primary and Secondary Central Nervous System Lymphoma

Primary and secondary central nervous system (CNS) lymphomas are aggressive brain tumors that pose an immense challenge to diagnose, to define in terms of molecular pathogenesis, as well as to effectively treat. During the past ten years improvements in survival have been achieved with the implementation of anti-CD20 immunotherapy and optimization of dose-intensive consolidation strategies. Nevertheless, a plateau has likely been achieved with existing genotoxic strategies, and an emerging set of targeted therapeutic and immunotherapeutic strategies have recently been evaluated and show activity in relapsed disease.

Dr. James Rubenstein will review the biology of primary and secondary CNS lymphoma, the challenges in diagnosis and in radiographic assessment, tumor genetics, and pro-survival signaling pathways. He will also review the results of recent early phase investigations of novel targeted agents and immunotherapeutic strategies in these diseases and discuss their limitations and potential in future investigations.

Dr. Andres Ferrari will discuss the standard of care strategies for primary CNS lymphoma, dose-intensive consolidation strategies including autologous stem cell transplant, the pros and cons of current agents, the problem of the blood-brain barrier and treatment related toxicity, results from recent and ongoing trials, and how to incorporate novel agents as part of first-line treatment.

Dr. Kerry Savage will review the clinical challenge of CNS relapse of systemic lymphoma. The CNS IPI risk model that identifies patients at high risk for CNS lymphoma, as well as emerging biomarkers of risk and differential therapeutic strategies based on brain compartment and age will be discussed. Novel therapeutic strategies to explore in the prophylaxis of CNS relapse will also be reviewed.