Lymphomas

Abstract Lymphoma can involve the nervous system either primarily or as a secondary complication of systemic lymphoma. This chapter is focused on primary nervous system lymphomas. Primary diffuse large B-cell lymphoma of the central nervous system (primary central nervous system lymphoma) is the most common primary lymphoma of the nervous system in adults and children and is recognized as a distinct pathological entity by the World Health Organization. Low-grade B-cell, Burkitt, T-cell, and T/NK-cell histotypes account for 10% or less of primary nervous system lymphomas. Initial evaluation of patients with primary central nervous system lymphoma involves assessment of the brain, eyes, cerebrospinal fluid, bone marrow, and extraneural organs. Treatment regimens for primary central nervous system lymphoma are centered around chemotherapy with or without whole brain irradiation, agents are included based on their ability to cross the blood–brain barrier, and based on patient age and comorbidities. Neurotoxicity of treatment is a significant concern, especially among elderly patients. Novel therapies are under development.