Primary lymphoma of the brain
Primary lymphoma of the brain is cancer of white blood cells that starts in the brain.
Brain lymphoma; Cerebral lymphoma; Primary lymphoma of the central nervous system; PCNSL; Lymphoma - 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:
Exams and Tests
The following tests may be done to help diagnose a primary lymphoma of the brain:
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/AIDS, may also be tried.
You and your health care provider may need to manage other concerns during your treatment, including:
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:
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.
National Cancer Institute website. Primary CNS lymphoma treatment (PDQ) – health professional version. www.cancer.gov/cancertopics/pdq/treatment/primary-CNS-lymphoma/HealthProfessional. Updated January 5, 2017. Accessed March 22, 2018.
National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology (NCCN guidelines): central nervous system cancers. Version 1.2017. www.nccn.org/professionals/physician_gls/pdf/cns.pdf. Updated August 18, 2017. Accessed March 22, 2018.
Review Date: 1/19/2018
Reviewed By: Richard LoCicero, MD, private practice specializing in hematology and medical oncology, Longstreet Cancer Center, Gainesville, GA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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