Primary CNS lymphoma (PCNSL) is a nervous-system-seeking extranodal non-Hodgkin's lymphoma that shares common morphologic and molecular features with diffuse large B-cell lymphomaLymphomagenesis is probably promoted through direct transforming properties of infectious agents (Epstein–Barr virus in immunocompromised patients) or sustained antigenic stimulation by infection or autoimmune diseasePCNSL presents with syndromes ranging from language deficits, paresis of extremities, brain swelling, brainstem dysfunction, endocrine dysregulation and behavioral abnormalities to peripheral neuropathies and signs of vitreous diseaseDiagnosis of PCNSL requires histopathological confirmation; clonality assessment by immunoglobulin gene rearrangement analysis or flow cytometry might prove useful not only for initial diagnosis but also for detection of minimal residual diseaseHigh-dose methotrexate is an essential component of any treatment regimen for PCNSL
Primary CNS lymphoma (PCNSL) is a nervous-system-seeking extranodal non-Hodgkin's lymphoma (NHL). Microscopically, PCNSL resembles systemic diffuse large B-cell lymphoma (DLBCL) and the two disorders share common molecular features; however, the underlying molecular mechanisms of PCNSL, including the causative role of somatic gene alterations, remain uncertain. Survival and functional outcome in patients with PCNSL have improved markedly since the deferral of cranial irradiation and the provision of methotrexate-based chemotherapy became standard of care, but most patients relapse, and the 5-year survival rate remains inferior to that of patients over 60 years of age with DLBCL outside the nervous system. This Review highlights advances in our understanding of PCNSL, as well as current approaches to its diagnosis and therapy.
