Primary CNS lymphoma: update on molecular pathogenesis and therapy

Abstract

Primary central nervous system lymphoma (PCNSL) is a rare and aggressive form of extra-nodal non-Hodgkin lymphoma that as a brain tumor poses a unique set of challenges in diagnosis and management. With the advent of next-generation sequencing, we review updates in the understanding of its molecular and genomic pathogenesis. We also highlight key issues in management, with a focus on emerging technologies and new biological therapies including monoclonal antibodies, IMiDs, BTK inhibitors, PD-1 inhibitors, and CAR-T therapy. Integration of these approaches will likely enhance induction and consolidation strategies to suppress NF-κB activation and the anti-tumor immune response, while minimizing the often noxious effects of genotoxic approaches.

Keywords:

• Primary CNS lymphoma
• brain tumor
• NF-κB
• IMiDs
• Bruton’s tyrosine kinase

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

Supported by NIH R01CA139-83-01A1.