Primary lymphoma of 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

• Changes in speech
• Changes in vision
• Confusion
• Fever
• Hallucinations
• Headaches
• Leaning to one side when walking
• Loss of coordination
• Numbness to hot, cold, and pain
• Personality changes
• Seizures
• Weakness in hands
• Weight loss

Exams and Tests

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

• Biopsy of the brain
• Head CT scan or MRI
• Spinal tap (lumbar puncture) to perform tests such as:
  • CSF cell count
  • CSF cytology
  • CSF EBV viral load
  • CSF flow cytometry
  • CSF total protein

Treatment

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.

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, 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

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: 2/7/2012

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.