Last Updated: September 28, 2018
Central nervous system (CNS) involvement with lymphoma is categorized into primary and secondary subtypes. These are vastly different clinical entities and therefore will be discussed separately in this chapter.
Primary CNS lymphoma (PCNSL) is an uncommon subtype of extranodal non-Hodgkin lymphoma involving the brain, leptomeninges, eyes, or spinal cord without systemic disease. PCNSL is seen annually in approximately 3% of patients diagnosed with primary brain tumors. These tumors represent about 1% of all non-Hodgkin Lymphomas and have an annual incidence of 0.47 per 100,000 person/year. PCNSL tends to present around 50-60 years of age in immunocompetent individuals and around 35 years of age in immunocompromised individuals.
Immunocompetence of the patient is a differentiating feature...