DOI: 10.1097/01.CNE.0000473751.26279.54
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Issn Print: 0163-2108
Publication Date: 2015/06/30
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Excerpt
Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin tumor of the brain, which originates in hematopoietic-extranodal tissue and is highly malignant. It was described initially by Bailey in 1929, who called it “perivascular sarcoma” because of the arrangement of tumor cells around the vessels. In 1938, this tumor was named “reticular cells sarcoma” by Yuile and then “microglioma” by Russel in 1948. The classification of this tumor as a different nosologic entity was not established until 1974 by Henry, and the current name was adopted by Rappaport.
As a rare form of extranodal lymphoma confined to the brain, PCNSL represents 2% to 6% of all intracranial neoplasms. Most PCNSLs originate in B-cells (92%–98%), and only rare cases are associated with T-cells. It is the most common tumor found in patients with AIDS; however, there have been an increasing number of immunocompetent patients presenting with this tumor, in whom the clinical course, histologic findings, and radiologic appearance are very different.
