Primary CNS lymphoma

Jai Grewal MD (Dr. Grewal of the Long Island Brain Tumor Center at Neurological Surgery, PC, received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Harpreet K Grewal MD (Dr. Grewal of JGMDPC in Roslyn, New York, has no relevant financial relationships to disclose; Dr. Grewal's spouse received research funding from Northwest Biotherapeutics and consulting fees from Novocure.)
Edward J Dropcho MD, editor. (Dr. Dropcho of Indiana University Medical Center has no relevant financial relationships to disclose.)
Originally released February 14, 1994; last updated October 12, 2014; expires October 12, 2017

This article includes discussion of primary CNS lymphoma, microglioma, NHL-CNS, non-Hodgkin lymphoma-central nervous system, PCNSL, perithelial cell sarcoma, perivascular sarcoma, and reticulum cell sarcoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Primary central nervous system lymphoma is a 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 authors discuss recent 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 should consider spinal, leptomeningeal, and ocular involvement.

 

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

 

• Treatment options include systemic chemotherapy, intra-CSF chemotherapy, radiotherapy, and targeted therapies.

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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