Primary lymphoma of the brain

Answer 1

Definition

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

Alternative Names

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

Causes, incidence, and risk factors

The cause of primary brain lymphoma is unknown. 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) infection, the virus that causes mononucleosis.

The rate of primary brain lymphoma is rising, but it is still relatively 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 performed to help diagnose a primary lymphoma of the brain:

• Biopsy of the brain
• CSF cell count
• CSF total protein
• Head CT scan or MRI

Treatment

The condition is usually first treated with corticosteroids to control any local 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 used to be the main treatment for primary lymphoma of the brain. Now it is usually reserved for treating patients who do not respond to chemotherapy.

Treatment with multiple therapies (combination therapy) is common.

Expectations (prognosis)

The survival of untreated primary brain lymphoma is under 2 months. Treated with chemotherapy, patients 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

References

DeAngelis LM. Tumors of the central nervous system and intracranial hypertension and hypotension. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 199.

National Cancer Institute. Primary CNS lymphoma treatment (PDQ). 2009. Accessed February 25, 2009.

Answer 2

Definition

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

Alternative Names

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

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.

Reviewed By

Review Date: 02/28/2011

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