Primary central nervous system lymphoma is a tumor of a form of white blood cells called lymphocytes. While lymphomas can occur anywhere in the body and often spread to the nervous system, primary central nervous system lymphoma refers to a form of lymphoma that is confined to the brain, spinal cord, their lining, and/or the inside of the eye.
While primary central nervous system lymphoma is sensitive to radiation therapy, long-term response is rare when radiation therapy is applied as the first treatment. However, use of a chemotherapy agent called methotrexate prior to radiation therapy has been shown to be effective at prolonging treatment responses. Interestingly, the addition of other forms of chemotherapy active in the treatment of lymphoma elsewhere in the body has not proven to be any more effective (though less safe) than methotrexate alone.
Treatment for disease that has recurred after radiation therapy consists of the use of high doses of chemotherapy, and is currently under investigation.
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