ABSTRACT
Primary central nervous system lymphoma, a rare form of extranodal lymphoma, occurs in the brain, leptomeninges, spinal cord, or eyes; typically, it remains confined to the central nervous system (CNS), and accounts for 3.1% of all primary brain tumors. Diffuse large B-cell lymphoma is the most common type of primary central nervous system lymphomas (PCNSL); the remaining 10% of cases are poorly characterized low-grade lymphomas, Burkitt’s lymphomas, and T-cell lymphomas. Gene expression studies have demonstrated three gene ‘signatures’ associated with PCNSL: germinal center B-cell, activated B-cell, and type 3 large B-cell lymphomas. Cerebrospinal fluid sampling should be considered in every patient with suspected or confirmed PCNSL; however, increased intracranial pressure may be a relative contraindication to lumbar puncture. Identification of prognostic factors enables physicians to discuss prognosis with individual patients, may eventually allow the application of risk-adjusted therapeutic strategies, and is critical for prospective study designs.
