Primary CNS lymphoma: update on molecular pathogenesis and therapy. Issue 1 (2nd January 2023)

• Record Type: Journal Article
• Authors: Mo, Shirley S.; Cleveland, Joseph; Rubenstein, James L.
• Is Part Of: Leukemia & lymphoma. Volume 64:Issue 1(2023)
• Page Start: 57
• Page End: 65
• Publication Date: 2023-01-02
• Subjects: Primary CNS lymphoma -- brain tumor -- NF-κB -- IMiDs -- Bruton's tyrosine kinase; Leukemia -- Periodicals; Lymphomas -- Periodicals; 616.99419
• DOI: 10.1080/10428194.2022.2133541
• Languages: English
• ISSNs: 1042-8194

Abstract

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.