Primary central nervous system lymphoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Primary CNS lymphomas are extranodal, malignant non-Hodgkin lymphomas of the diffuse large B-cell type that are confined to the brain, eyes, leptomeninges, or spinal cord, in the absence of systemic lymphoma. Primary CNS lymphomas are estimated to account for up to 1% of all lymphomas, 4–6% of all extranodal lymphomas, and about 3% of all CNS tumours. After a continuous rise in the incidence of primary CNS lymphoma during the 1980s and 1990s, epidemiological data in high-income countries show a decrease in incidence, particularly among young patients with AIDS.

Classification

Primary central nervous system lymphoma may be classified according to the site of involvement of the central nervous system into 3 groups:

• Parenchymal

• Non-Hodgkin B cell type / diffuse large B-cell non-Hodgkin's lymphoma (most common)
• Non-Hodgkin T cell type
• Primary CNS involvement with Hodgkin disease

• Intravascular lymphoma
• Primary leptomeningeal lymphoma

• Primary dural lymphoma