CNS Lymphoma

What is CNS lymphoma?

CNS lymphoma is a rare, aggressive cancer that develops in your central nervous system (CNS). Tumors may form in your brain, spinal cord, spinal fluid and (as it’s so close to your brain) your eye. It’s a type of non-Hodgkin lymphoma.

CNS lymphoma starts in white blood cells called lymphocytes, which are part of your lymphatic system. Your lymphatic system is an important part of your immune system. It helps your body fight infections and diseases. When the lymphoma starts in your CNS and isn’t found anywhere else in your body, it’s called primary CNS lymphoma. If lymphoma is in other parts of your body, as well as in your CNS, it’s called secondary CNS lymphoma.

Although anyone can get this cancer, you’re more likely to be diagnosed if you’re a person assigned male at birth (AMAB) who has a weakened immune system from a condition like HIV and AIDS. People over 65 are also more at risk. Still, this cancer is extremely rare. Only about 1,500 new cases are diagnosed in the U.S. each year.

No matter your unique situation, your healthcare team will work with you to find the right treatment to fight CNS lymphoma.

What are the symptoms of CNS lymphoma?

Symptoms depend on where the tumor is located. For instance, CNS lymphoma may not cause symptoms if it’s in the membrane covering your brain and spinal cord (meninges). But a tumor near your eyes often causes vision changes. If the mass occurs near the area of your brain that controls movement, you could have weakness or coordination changes.

Symptoms of CNS lymphoma may include:

• Nausea and vomiting
• Weakness in your arms, legs or face
• Weakness affecting one side of your body (hemiparesis)
• Hearing loss
• Difficulty swallowing (dysphagia)
• Signs of brain pressure (headaches, confusion)
• Vision problems (blurry vision, seeing double, floaters)
• Changes in your mental state (trouble speaking, memory loss, feeling sluggish)
• Seizures (that may become more frequent over several days or weeks)
• Trouble controlling when you pee or poop (urinary and fecal incontinence)

What causes CNS lymphoma?

Like other types of lymphoma, CNS lymphoma forms when cells in lymph tissue start to behave abnormally. They multiply out of control and overtake healthy cells. With CNS lymphoma, the cells that start growing abnormally are usually white blood cells (lymphocytes) called B cells.

Researchers aren’t sure what causes a lymphocyte to transform into a cancer cell. But they’ve identified factors that may increase your risk of CNS lymphoma.

Risk factors for CNS lymphoma

Certain conditions associated with having a weakened immune system may increase your risk of CNS lymphoma. Risk factors include:

• HIV/AIDS (especially if you have an active Epstein-Barr infection)
• Wiskott-Aldrich syndrome (WIS)
• Common variable immunodeficiency state (CVID)
• Ataxia-telangiectasia
• Taking immunosuppressant drugs following an organ transplant

How is CNS lymphoma diagnosed?

Your healthcare provider may recommend different procedures and tests to diagnose CNS lymphoma. Cancer staging also takes place during diagnosis. Cancer staging helps providers determine how advanced the cancer is and which treatments will likely work best.

You may need:

• Exams. Your provider will check the health of your brain, spinal cord and eyes. They’ll perform a neurological exam to check your CNS. They may do a slit lamp exam to find signs of a tumor behind your eye.
• Imaging tests. Your provider may order an MRI, CT scan or PET scan to see where cancer is located inside of your body. CNS lymphoma rarely spreads beyond your central nervous system, but it may spread quickly within it.
• Blood tests. Blood tests allow your provider to check your cells for signs of cancer. Tests include a complete blood count, blood chemistry study and an HIV test.
• Tissue and fluid tests. Your provider may remove a sample of fluid or tissue from your spinal fluid, bone marrow or the tumor itself to test for cancer cells.

How is CNS lymphoma treated?

Not everyone agrees on the best treatment for CNS lymphoma. Instead, your healthcare team will suggest a care plan based on various factors, like your age, your HIV/AIDS status and whether the cancer is newly diagnosed or recurrent (returned after treatment). Treatment will likely involve a combination of therapies.

Treatments for CNS lymphoma include:

• Chemotherapy. Chemotherapy uses drugs to kill cancer cells. High doses of a chemotherapy drug called methotrexate (HD-MTX) are often used to treat newly diagnosed CNS lymphoma.
• Radiation. Radiation uses beams of energy to kill cancer cells. One type of radiation treatment for symptom relief is whole-brain radiation. It destroys cancer cells throughout your brain.
• Targeted therapy. Targeted therapy uses substances like proteins and antibodies to attack cancer cells. Rituximab and ibrutinib are targeted therapy treatments that your provider may recommend.
• Stem cell transplant. During a stem cell transplant, you receive healthy blood cells to replace blood cells damaged during cancer treatments like chemotherapy and radiation.
• Clinical trial. Your provider may recommend you take part in a clinical trial to test new treatments. Current trials are studying the effectiveness of new targeted therapy drugs and new combinations of chemotherapy drugs in CNS lymphoma treatment.

If you’re HIV-positive or have AIDS, you’ll continue antiretroviral therapy (ART) while you receive treatments for CNS lymphoma.

Complications/side effects of treatment

Treatments for CNS lymphoma can cause side effects that your healthcare provider will discuss with you beforehand. For example, whole-brain radiation destroys cancer cells in your brain. But it may also lead to several severe side effects that can impact your brain function. All treatment options pose potential risks.

Discuss the benefits and risks of your treatment plan with your provider. Ask if they recommend palliative care. This treatment can help you manage side effects and the overall impact cancer has on your life.

What can I expect if I have this condition?

CNS lymphoma is a fast-spreading cancer that often returns following treatment. Still, your prognosis (chance of recovery) depends on several unique factors, including:

• Your age
• Your overall health
• Your HIV status
• The location of the tumor
• The result of your blood chemistry studies (which can tell how the cancer may be impacting your organs)

Your healthcare team will work with you to find the treatment plan that gives you the best chance of survival without sacrificing your quality of life.

What is the survival rate for CNS lymphoma?

Researchers report on cancer survival rates by tracking how many people with a certain cancer diagnosis are alive after a set time, usually five years. The five-year survival rate for people with CNS lymphoma is 30%. This means that 3 out of 10 people diagnosed with CNS lymphoma are alive five years later.

Still, these numbers are general. They don’t factor in other specifics that affect prognosis. For instance, outcomes are usually better if you’re not immunocompromised or if the lymphoma hasn’t spread beyond your brain. Survival rates also don’t consider the impact that new treatments may have on your life expectancy.

Your provider is your best resource for explaining how your health and unique cancer diagnosis will shape your likely outcome.

How do I take care of myself?

Living with lymphoma is hard. You may feel anxious about what tests or treatments lie ahead. You may not know how to share what you’re feeling with others.

Now, more than ever is the time to reach out and take advantage of every available resource. This may mean reaching out to loved ones, even if it’s difficult. It may mean asking your healthcare provider about palliative care or support groups. Everyone’s cancer journey is different. But it’s essential to connect with others every step of the way.

When should I see my healthcare provider?

Even when CNS lymphoma goes into remission (no signs or symptoms of cancer), you’ll need regular check-ups to see if the cancer comes back. You’ll need more frequent visits within the first five years of treatment. Most CNS lymphoma that recurs comes back within the first five years.

What questions should I ask my healthcare provider?

Questions you may want to ask include:

• What tests will I need to determine my care plan?
• What are the risks and benefits associated with various treatment options?
• How often will I need follow-up testing to see how the cancer responds to treatment?
• Am I eligible to enroll in a clinical trial? Would you recommend I do so?
• Do you recommend palliative care?