| Abstract: |
Primary central nervous system lymphoma is a rare disease with an annual incidence rate of seven cases per 1,000,000 people in the USA. More than 90% of patients with primary central nervous system lymphoma display a diffuse large B cell lymphoma phenotypic subtype. Systemic workup is needed before starting therapy to rule out systemic involvement. Methotrexate-based chemotherapy regimens achieve a high response rate and improved survival. The role of whole-brain radiotherapy is less well defined and may prolong progression-free survival but is also associated with progressive cognitive deterioration and dementia. New therapeutic strategies such as adding rituximab to methotrexate-based chemotherapy, and consolidation with high-dose chemotherapy with autologous stem cell transplant, have shown promising results. © 2017 John Wiley & Sons Ltd. |
