Primary lymphoma of the brain

Primary lymphoma of the brain is cancer of the lymph cells that starts in the brain.

Causes

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:

  • HIV
  • Organ transplants (especially heart transplants)

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.

Symptoms

Exams and Tests

The following tests may be done to help diagnose a primary lymphoma of the brain:

Treatment

The condition is usually first treated with corticosteroids to control swelling and improve symptoms. However, chemotherapy may increase survival by 3 - 4 years, or longer. The chemotherapy is usually high doses of methotrexate given through a vein (intravenously) or a spinal tap (intrathecally).

Treating patients with weakened immune systems is not as successful, but it is improving.

Radiation therapy 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 treatment. This combination therapy includes drugs such as temozolomide, rituximab, cytarabine, and etoposide.

Recently, younger patients have been treated with high doses of chemotherapy. After the chemotherapy, they have an autologous stem cell transplant.

Outlook (Prognosis)

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.

About 40% of patients are alive at 5 years. In general, older patients have a worse outlook than younger patients.

Possible Complications

Possible complications include:

  • Chemotherapy side effects, including low blood counts
  • Radiation side effects, including confusion, headaches, nervous system (neurologic) problems, and tissue death
  • Return (recurrence) of the lymphoma

Alternative Names

Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; Lymphoma - brain

References

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.

Update Date: 2/28/2012

Reviewed by: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital.

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