Vol 11, No 6 (2015)
Review paper
Published online: 2016-03-08
Primary central nervous system lymphoma — a review of current therapeutic strategies
Oncol Clin Pract 2015;11(6):310-316.
Abstract
Primary central nervous system lymphoma (PCNSL) is a highly aggressive extranodal lymphoma subtype arising in the brain parenchyma, spinal cord, meninges, cranial nerves, and/or intraocularly. PCNSL accounts for 3% of brain tumours and 2–3% cases of non-Hodgkin’s lymphoma. Diffuse large B-cell lymphoma (DLBCL) is a primary diagnosis in 95% of all PCNSL, with highly aggressive lymphomas (Burkitt’s lymphoma, lymphoblastic lymphoma) and marginal zone lymphoma (MZL) or T-cell lymphomas accounting for the other 5%. Over the last 40 years, PCNSL rates have been increasing. Although PCNSL shares many histopathological features with DLBCL (not otherwise specified; NOS), its prognosis is generally far worse. The current WHO 2008 classification for cancers of the haematopoietic system and lymphomas assigns DLBCL CNS into a distinct diagnostic entity of lymphoma.
Keywords: llymphomacentral nervous systemmethotrexate
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