Primary CNS lymphoma is a rare type of lymphoma that starts in the central nervous system (CNS) — brain, spinal cord, and eyes.¹ If you or a loved one has been diagnosed with primary CNS lymphoma, it’s important to get the facts about this condition and find out what to expect from treatment.

What Is Primary CNS Lymphoma?

About 4 in 1 million people get primary CNS lymphoma, sometimes referred to as PCNSL.¹ People diagnosed with primary CNS lymphoma often have weakened immune systems. Your risk of developing primary CNS lymphoma goes up with age.¹ The median age at diagnosis of PCNSL is 65 years.

In some people, lymphoma develops in another part of their body and later spreads to the brain, spinal cord, or eyes. This is known as secondary CNS lymphoma.²

What Are the Symptoms?

Primary CNS lymphoma can cause a wide range of symptoms. The most common symptoms are related to problems with thinking and changes in the way a person behaves. These symptoms may appear gradually over weeks or months. Symptoms might include^3,4:

• Speech problems
• Trouble remembering things
• Confusion
• Weakness or paralysis on one side of the body
• Changes in personality
• Seizures
• Depression or apathy
• Hallucinations (seeing things that aren’t there)
• Abnormally slow movement
• Headaches
• Vomiting
• Nausea
• Blurred vision or floaters (small spots or threads that appear to drift across your field of vision)

When primary CNS lymphoma develops, symptoms may begin years before lymphoma is diagnosed.¹

How Is It Diagnosed?

Only a healthcare provider can diagnose primary CNS lymphoma, so it’s important to discuss your symptoms with them. If your doctor suspects you have primary CNS lymphoma, they will likely recommend you get a magnetic resonance imaging (MRI) scan. This imaging test will allow them to look for changes in your brain. They may also recommend a positron emission tomography (PET) scan to make sure you don’t have signs of cancer elsewhere in your body.¹ Diagnosis is typically confirmed with a biopsy or by testing cerebrospinal fluid collected by a lumbar puncture (sometimes called a spinal tap).⁵

Initial Treatment Options

Your treatment options for primary CNS lymphoma can depend on many factors, including your age, your overall health, any additional health conditions you have, and your priorities. Doctors often recommend chemotherapy as a first step. For people who can’t tolerate chemotherapy, doctors may recommend radiation therapy or additional therapies that might have less harsh side effects. Younger people with primary CNS lymphoma may be eligible to receive a bone marrow transplant after chemotherapy.¹

About one-third of people have primary CNS lymphoma that is refractory to chemotherapy, meaning the treatment does not work well for them.⁶ For people whose primary CNS lymphoma does respond to chemotherapy, up to 60 percent will eventually relapse.⁶ Most relapses happen within the first two years.⁷

Relapsed or Refractory Treatment Options

There are currently no treatments approved by the U.S. Food and Drug Administration (FDA) specifically for people with relapsed or refractory primary CNS lymphoma. As a result, most doctors recommend that people with relapsed or refractory primary CNS lymphoma consider joining a clinical trial.¹ Enrolling in a clinical trial can give you access to investigational treatments currently being studied. However, beyond suggesting clinical trials, your doctor may recommend additional options, including other medications that work in different ways.

Prognosis and Survival Rates

For people who are still in remission after five years, the prognosis improves. Relapses become less common after five years but are still possible. People have experienced relapse after 10 years.¹

The prognosis of people who have a relapse of primary CNS lymphoma and those who are refractory to treatment is poor. The one-year overall survival rate is 38 percent. That means 38 percent of people will still be alive after one year. At three years, the overall survival rate is 25 percent.¹

Get Support From Others Who Understand

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