Abstract
Primary central nervous system lymphoma (PCNSL) is defined as lymphoma confined to the central nervous system (CNS) at presentation. PCNSL is a rare, aggressive extranodal non-Hodgkin lymphoma (NHL) that is confined to the CNS with no evidence of prior or current systemic disease. It may involve the brain, eyes, leptomeninges, or the spinal cord. The typical clinical presentation of PCNSL involves progressive and relatively rapid focal neurological symptoms associated with the neuroanatomic localization of the tumor. MRI with contrast is the most sensitive imaging modality for the detection of PCNSL. Over 90% of cases of PCNSL are diagnosed histologically as diffuse large B-cell lymphoma (DLBCL). Current therapy consists of a methotrexate-based combination chemotherapy with rituximab for B-cell lymphomas. Consolidation treatment varies according to the characteristics of the patient and is best conducted by a multidisciplinary team. More than half of PCNSL patients who respond to treatment suffer from relapse. Current options include chemotherapy, immunotherapy, radiotherapy, or enrollment in a clinical trial. Treatment-related neurotoxicity is a major complication of PCNSL treatment and requires frequent follow up after treatment.
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