Abstract: |
Primary central nervous system lymphoma (PCNSL) is an uncommon, extranodal non-Hodgkin lymphoma that involves the brain primarily, with or without involvement of the leptomeninges, eyes, or spinal cord, in the absence of systemic (non-CNS) disease. It tends to affect elderly or immunocompromised individuals. Early studies of whole-brain radiation therapy (WBRT) alone revealed a robust initial response but high rates of local recurrence and disease-related mortality. The introduction of high-dose methotrexate-based chemotherapy prior to RT improved the durability of disease control and the rates of survival. However, late neurotoxicity emerged as an important complication, particularly for elderly patients. Recent research has focused on approaches to maintain improved survival outcomes while minimizing the toxicity of therapy. These include selective WBRT dose reduction and substitution of WBRT with other consolidation strategies. This chapter explores the diagnosis, prognosis, and treatment of PCNSL, with an emphasis on the evolving role of WBRT in its management and discussion of emerging contemporary treatment options including adoptive cellular therapies. © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2018, 2024. |