Abstract
This chapter presents a discussion on primary and secondary lymphoma of the central nervous system (CNS). Patients with malignant lymphoma frequently have nervous system involvement. This can take the form of primary central nervous system lymphoma (PCNSL) or metastatic disease from systemic lymphoma. Both primary and metastatic lymphomas are almost always non-Hodgkin's lymphomas (NHL) as Hodgkin's disease is rare. PCNSL is usually located in the basal ganglia, corpus callosum or cerebellum and is commonly in contact with either the ependyma of the ventricles or the subarachnoid space. Periventricular growth of PCNSL in the absence of focal masses has also been described at autopsy. In immunosuppressed patients with PCNSL, Epstein-Barr virus (EBV) can be detected in the CSF or in parenchymal tumor tissue in all patients. In these patients, PCNSL develops from an EBV-driven B-cell clone that comes from latently infected immortalized B-cells whose proliferation is normally controlled by Tcell-mediated immunity. The chapter discusses primary central nervous system lymphoma, secondary CNS manifestation of systemic non-Hodgkin's lymphoma, CNS manifestation of systemic Hodgkin's lymphoma.
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