Primary lymphoma of the brain is cancer of white blood cells that starts in the brain.
The cause of primary brain lymphoma is not known. It is more common in people ages 45 - 70.
Patients who have a weakened immune system are at greater risk for primary lymphoma of the brain. Common causes of a weakened immune system include:
Primary lymphoma of the brain is also linked to Epstein-Barr Virus (EBV), the virus that causes mononucleosis, especially in people with HIV infection.
The rate of primary brain lymphoma is rising. However, this cancer is still rare.
The following tests may be done to help diagnose a primary lymphoma of the brain:
The condition is usually first treated with corticosteroids to control swelling and improve symptoms. The main treatment is with chemotherapy. The chemotherapy is usually high doses of methotrexate given through a vein (intravenously) or a spinal tap (intrathecally).
Treating patients with a weakened immune system (such as those with HIV) is not as successful, but it is improving.
Radiation therapy of the whole brain was once the main treatment for primary lymphoma of the brain. Now it is usually only given to patients who do not respond to chemotherapy.
Many patients receive more than one chemotherapy drug. These combination therapies include drugs such as temozolomide, rituximab, cytarabine, and etoposide.
Recently, younger patients have been treated with high doses of chemotherapy, followed by an autologous stem cell transplant. Clinical trials are studying the best treatment to give after the first chemotherapy.
Without treatment, patients with primary brain lymphoma survive for less than 2 months. Patients who are treated with chemotherapy often survive 3 - 4 years or more, depending on whether the tumor stays in remission.
About 40% of patients are alive at 5 years. Survival may improve with the increased use of autologous stem cell transplants. In general, older patients have a worse outlook than younger patients.
Possible complications include:
Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain
National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Non-Hodgkin's Lymphoma. v.1.2011. Accessed February 8, 2011.
National Cancer Institute. Primary CNS lymphoma treatment (PDQ). 2009. Accessed February 25, 2009.
Updated by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.
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