Primary Cerebral Lymphoma (PCL)
Primary cerebral lymphoma is a rare cancer that starts in the lymph tissues of the brain or spinal cord. It is also known as brain lymphoma or central nervous system lymphoma.
The brain and spinal cord make up the central nervous system (CNS). Cells called lymphocytes are part of the lymph system and can travel through the CNS. When lymphocytes become cancerous they can cause cancer in these tissues.
The cancer is called primary cerebral lymphoma when it starts in the CNS. It can also start in the eye. When it spreads to the brain it’s called secondary cerebral lymphoma.
Without treatment, primary cerebral lymphoma can be fatal within one to three months. If you receive treatment, some studies have shown 70 percent of people are still alive five years after treatment.
The cause of primary cerebral lymphoma is unknown. But since lymph tissues are part of the immune system, people with impaired immune systems are at increased risk of developing this form of cancer. It’s also been associated with the Epstein-Barr virus.
Advanced HIV infection, also known as AIDS, and other immune system dysfunctions are risk factors for developing primary cerebral lymphoma. HIV can be spread through sexual contact, sharing needles, or contaminated blood and blood products. Without treatment the virus attacks and destroys the immune system.
In AIDS, the body’s immune system is weakened and becomes vulnerable to a variety of infections and cancers like primary cerebral lymphoma.
Other risk factors for developing primary cerebral lymphoma include having an organ transplant, having an autoimmune disease, or being older than age 50. The older you are, the more susceptible your body is to this disease.
Symptoms of primary cerebral lymphoma include:
- changes in speech or vision
- nausea and vomiting
- difficulty walking
- changes in personality
- paralysis on one side of the body
Not everyone has the same symptoms or has every symptom. In order to receive an accurate diagnosis, your doctor needs to run a variety of tests.
Your doctor will ask you questions about your symptoms along with your medical and family history. They’ll also perform a physical exam that includes an evaluation of your neurologic system like your mental status, balance, and reflexes. In this exam, you may be asked to speak, use basic motor functions like pushing and pulling, and watch and respond to your doctor’s finger movements.
Other tests used to diagnose primary cerebral lymphoma include:
- CT scan
- blood work
- slit lamp exam, in which your doctor looks at the structure of your eyes with a specialized instrument to check for abnormalities
- lumbar puncture (spinal tap), in which a needle is inserted between two of the vertebrae in your low back in order to draw out a sample of cerebrospinal fluid.
The methods used to treat primary cerebral lymphoma depend on:
- the severity and extent of the cancer
- your age and health
- your expected response to treatment
Your doctor will talk to you about your treatment options and what to expect regarding side effects. Treatment options include:
Radiation uses high-energy rays to shrink and kill cancer cells. In primary cerebral lymphoma, whole brain radiation was one of the first treatments used. Now due to more effective treatment options, radiation therapy is combined with chemotherapy. Radiation is rarely used alone when treating this type of cancer.
There are also promising studies on Gamma Knife Radiosurgery (GKRS). This treatment is not surgery. It’s a precise delivery system of radiation. GKRS can benefit patients when combined with chemotherapy.
Chemotherapy is the use of drugs to kill cancer cells. Instead of killing the cells in one area, it’s a systemic treatment. This means that the drugs are able to go through the bloodstream to other parts of your body. Chemotherapy drugs can be given orally or intravenously.
Typically, a combination of chemotherapy drugs is used together when treating primary cerebral lymphoma. Older studies have shown chemotherapy for this cancer was not well tolerated in older adults. Newer studies on newer combinations of drugs show more promising results.
The body naturally makes steroid hormones. Synthetic steroids have powerful anti-inflammatory properties and can shrink lymphoma tumors. These steroids are generally safe at any age.
Clinical trials use new medications or treatments to see if they are more effective than current treatments. Trials are closely monitored, and you can leave a trial at any time. But they are not appropriate for everyone. If you’re interested in participating in a clinical trial, talk to your doctor about possible options.
Stem cell transplants
Your doctor may suggest transplanting your own healthy stem cells into your blood to help your body re-grow blood cells. This is called autologous stem cell transplantation.
The healthy cells are collected from your body after a course of high-dose chemotherapy. Clinical trials are currently underway to see how this procedure can benefit people with primary cerebral lymphoma.
Your doctor may suggest using certain drugs to help get rid of cancer cells without damaging healthy cells. This can be done with other treatments like radiation therapy.
In targeted therapy, antibodies created from an immune cell are infused into your body to destroy cancer cells or prevent them from growing and spreading. Rituximab is one example of a drug used in targeted therapy for primary cerebral lymphoma.
Side effects vary depending on your treatment. For example, radiation can cause neurological complications, headaches, and confusion. Chemotherapy causes a variety of side effects depending on the drugs used, including:
- nausea and vomiting
- nerve damage
- hair loss
- mouth sores
Talk with your doctor about what to expect and how to minimize any side effects.
Complications can arise due to the cancer or to the side effects of treatment, especially in the case of chemotherapy. These complications can include:
- low blood count
- brain swelling
- relapse, or the return of symptoms after treatment
- loss of neurological function
Primary cerebral lymphoma has a recurrence rate of 35 to 60 percent. Recent studies have shown that the five-year survival rate is 70 percent. This rate is likely to increase as new treatments and treatment combinations are discovered.
Your overall recovery and outlook depend on several factors, including:
- your age
- your health
- how advanced your condition is
- the extent to which lymphoma has spread
- how well you can function on a daily basis without help
The earlier you’re diagnosed, the more likely you are to receive effective treatment, extend your survival, and improve your quality of life while living with cancer.
Written by: Jaime Herndon and Tim Jewell
Medically reviewed on Jan 04, 2017 by Judith Marcin, MD