CNS lymphoma, or primary CNS lymphoma, is a rare cancer that affects your central nervous system (CNS). The cancer may form in your brain, spinal cord, spinal fluid or behind your eye. It can spread throughout your CNS quickly. Treatment options vary depending on your circumstances.
CNS lymphoma is a rare type of cancer that develops in your central nervous system (CNS). It 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.
When the lymphoma starts in the CNS and isn’t found anywhere else in your body, it’s called primary CNS lymphoma. If lymphoma is found in other parts of your body, as well as your CNS, it’s called secondary CNS lymphoma.
CNS lymphoma starts in white blood cells called lymphocytes that 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.
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CNS lymphoma is rare. There are only about 1,500 new cases of CNS lymphoma diagnosed in the United States each year. It occurs most often in people over 50, with the average age of diagnosis being 65. It’s slightly more common in men and people assigned male at birth (AMAB).
People with HIV, AIDS and other immunodeficiencies are more likely to receive a CNS lymphoma diagnosis.
Symptoms depend on where your tumor is located. A tumor is a mass of cancer cells. For instance, CNS lymphoma likely won’t cause symptoms if the tumor’s located in the membrane covering your brain and spinal cord (meninges). In contrast, a tumor near one or both 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:
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. Still, they’ve identified factors that may increase your risk of CNS lymphoma.
Certain conditions associated with having a weakened immune system may increase your risk of CNS lymphoma. They include:
Taking immunosuppressant drugs following an organ transplant may also increase your likelihood of developing certain cancers, including CNS lymphoma.
Your healthcare provider may recommend multiple procedures and tests to diagnose CNS lymphoma. Cancer staging also takes place during diagnosis. Cancer staging allows your provider to classify your cancer based on the tumor size, location and whether it’s spread beyond the site where the cancer started (metastasis).
Your provider will perform exams to check the health of your brain, spinal cord and eyes.
Your provider may order tests to see where cancer is located inside of your body. CNS lymphoma rarely spreads beyond your central nervous system, but it may spread quickly throughout various parts of your CNS.
Your provider may take a sample of tissue or fluid to be tested in a lab for cancer cells.
Various lab tests allow your provider to check your cells for signs of cancer.
There aren’t standard treatments for CNS lymphoma. Instead, your healthcare team will suggest a care plan based on various factors, including 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:
If you’re HIV-positive or have AIDS, you’ll continue antiretroviral therapy (ART) while you receive treatments for CNS lymphoma.
Your provider may recommend you take part in a clinical trial. A clinical trial is a study used to test new treatments for safety and effectiveness. Researchers are currently studying the potential of stem cell transplants and other therapies to more effectively treat CNS lymphoma. During a stem cell transplant, you receive healthy blood cells to replace blood cells that may be damaged during cancer treatments like chemotherapy and radiation.
CNS lymphoma is a fast-spreading cancer that often returns following treatment. Still, your prognosis depends on a variety of unique factors, including:
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 under 70 is 30%. The survival timeline for people older than 70 is six to seven months.
Still, these numbers are general. They don’t factor in other specifics that affect prognosis. For instance, outcomes are generally 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 lifespan.
Your provider is your best resource for explaining how your health and cancer will shape your care plan and likely outcomes.
A note from Cleveland Clinic
CNS lymphoma is a serious condition that requires close monitoring and a treatment plan designed especially for you. It’s important to work closely with a care team with experience treating lymphomas or cancers affecting the CNS. Your care team can explain your treatment options and help you weigh the benefits against potential risks or side effects. Depending on your cancer, they may recommend you participate in a clinical trial. They can connect you with palliative care to see to your comfort as you determine the next steps.
Last reviewed by a Cleveland Clinic medical professional on 07/05/2022.
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