Primary CNS lymphoma

Rimas V Lukas MD (

Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from Novocure for speaking engagements, honorariums from Novocure for advisory board membership, and research support from BMS.

Originally released February 14, 1994; last updated December 8, 2020; expires December 8, 2023


Primary central nervous system lymphoma is an extranodal non-Hodgkin lymphoma that arises within and is confined to the nervous system, including the brain, spinal cord, leptomeninges, and eyes. In this article, the author discusses developments in the management of primary central nervous system lymphoma.

Key points


• Primary central nervous system lymphoma is a non-Hodgkin (typically B-cell) lymphoma that arises within, and generally remains confined to, the nervous system.


• Staging work-up differentiates primary CNS lymphoma from extra-CNS lymphoma with CNS involvement and should consider spinal, leptomeningeal, and ocular involvement and exclude involvement outside of the nervous system.


• In the immunocompromised setting, the disease is often associated with the Epstein-Barr virus.


• Treatment options include systemic chemotherapy, intra-CSF chemotherapy, radiotherapy, and targeted therapies. High-dose methotrexate forms the backbone of many treatment protocols.

Historical note and terminology

Primary central nervous system lymphoma was first described in 1929 by Bailey (Bailey 1929). Over the following 2 to 3 decades, cerebral lymphoma was recognized as a discrete entity but described under a variety of names including reticulum cell sarcoma, histiocytic sarcoma, and microglioma, highlighting the debate regarding the malignant cell of origin; however, modern immunohistochemical studies definitively established the lymphocytic nature of this tumor.

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