What is the prevalence of brain and CNS lymphoma?

Updated: Feb 28, 2019
  • Author: Djamil Fertikh, MD; Chief Editor: James G Smirniotopoulos, MD  more...
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Lymphoma is the fifth most common cancer in the United States, with an annual incidence of 74,490 estimated cases. Approximately 88% of these cancers are non-Hodgkin lymphomas (NHLs). The incidence of NHL has doubled over the last 20 years because of the increase in AIDS-related lymphoma and the use of immunosuppressive drugs with organ transplantation and cancer chemotherapy. [1, 2, 3] Several rare congenital immunodeficiency syndromes may result in CNS lymphoma, including Wiskott-Aldrich syndrome, X-linked immunodeficiency, immunoglobulin A deficiency, and severe immunodeficiency syndrome. [4, 5, 6, 7, 8, 9, 10]

Primary lymphoma of the central nervous system is rare, representing about 1% of all lymphomas, less than 5% of all non-Hodgkin lymphoma, and 3-5% of brain tumors. [11, 12]

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