Primary lymphoma of the brain

Definition

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

Alternative Names

Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; PCNSL; Lymphoma - B-cell lymphoma, brain

Causes

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. Almost 1,500 new patients are diagnosed with primary brain lymphoma every year in the United States.

Symptoms

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

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

Exams and Tests

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

• Biopsy of the brain tumor
• Head CT scan, PET scan or MRI
• Spinal tap (lumbar puncture)

Treatment

Primary lymphoma of the brain is often 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/AIDS, 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

Outlook (Prognosis)

Without treatment, people with primary brain lymphoma survive for less than 6 months. When treated with chemotherapy, half of the patients will be in remission 10 years after being diagnosed. Survival may improve with autologous stem cell transplant.

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

Images

References

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

Grommes C, DeAngelis LM. Primary CNS lymphoma. J Clin Oncol. 2017;35(21):2410–2418. PMID: 28640701 pubmed.ncbi.nlm.nih.gov/28640701/.

National Cancer Institute website. Primary CNS lymphoma treatment (PDQ) - health professional version. www.cancer.gov/cancertopics/pdq/treatment/primary-CNS-lymphoma/HealthProfessional. Updated May 24, 2019. Accessed February 7, 2020.

National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology (NCCN guidelines): central nervous system cancers. Version 2.2020. www.nccn.org/professionals/physician_gls/pdf/cns.pdf. Updated April 30, 2020. Accessed August 3, 2020.