Definition of Primary lymphoma of the brain:

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

Causes, incidence, and risk factors:

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
• Numbness to hot, cold, and pain
• Personality changes
• Seizures
• Weakness in hands
• Weight loss

Signs 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 flow cytometry
  • CSF total protein

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.

Expectations (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.

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