Primary lymphoma of the brain

The cause of primary brain lymphoma is not known.

People with a weakened immune system are at high risk for primary lymphoma of the brain. Common causes of a weakened immune system include HIV/AIDS and having had an organ transplant (especially heart transplant).

Primary lymphoma of the brain may be linked to Epstein-Barr Virus (EBV), especially in people with HIV/AIDS. EBV is the virus that causes mononucleosis.

Primary brain lymphoma is more common in people ages 45 to 70. The rate of primary brain lymphoma is rising. But this cancer is still very rare.

Symptoms of primary brain lymphoma may include any of the following:

• Changes in speech or vision
• Confusion or hallucinations
• Fever
• Headaches or seizures
• Leaning to 1 side when walking
• Weakness in hands or loss of coordination
• Numbness to hot, cold, and pain
• Personality changes
• Weight loss

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)

Primary lymphoma of the brain is usually first treated with corticosteroids. These medicines are used to control swelling and improve symptoms. The main treatment is with chemotherapy.

Younger people may receive high-dose chemotherapy, possibly followed by an autologous stem cell transplant.

Radiation therapy of the whole brain may be done after chemotherapy.

Boosting the immune system, such as in those with HIV, may also be tried.

You and your health care provider may need to manage other concerns during your treatment, including:

• Having chemotherapy at home
• Managing your pets during chemotherapy
• Bleeding problems
• Dry mouth
• Eating enough calories
• Safe eating during cancer treatment

Without treatment, people with primary brain lymphoma survive for less than 2 months. Those treated with chemotherapy often survive 3 to 4 years or more. This depends on whether the tumor stays in remission. Survival may improve with autologous stem cell transplant.

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

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

Baehring JM, Hovhberg FH. Primary nervous system tumors in adults. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SK, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 74.

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: non-Hodgkin's lymphomas. Version 2.2016. www.nccn.org/professionals/physician_gls/pdf/nhl.pdf. Accessed March 17, 2016.

National Cancer Institute. PDQ primary CNS lymphoma treatment. Bethesda, MD: National Cancer Institute. Updated April 2, 2015. cancer.gov/cancertopics/pdq/treatment/primary-CNS-lymphoma/HealthProfessional. Accessed March 17, 2016.

Updated by: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.