General Information About Primary CNS Lymphoma
Primary central nervous system (CNS) lymphoma is a disease in which malignant (cancer) cells form in the lymph tissue of the brain and/or spinal cord.
Lymphoma is a disease in which malignant (cancer) cells form in the lymph system. The lymph system is part of the immune system and is made up of the lymph, lymph vessels, lymph nodes, spleen, thymus, tonsils, and bone marrow. Lymphocytes (carried in the lymph) travel in and out of the central nervous system (CNS). It is thought that some of these lymphocytes become malignant and cause lymphoma to form in the CNS. Primary CNS lymphoma can start in the brain, spinal cord, or meninges (the layers that form the outer covering of the brain). Because the eye is so close to the brain, primary CNS lymphoma can also start in the eye (called ocular lymphoma).
The lymph system is part of the body's immune system and is made up of tissues and organs that help protect the body from infection and disease. These include the tonsils, adenoids (not shown), thymus, spleen, bone marrow, lymph vessels, and lymph nodes. Lymph tissue is also found in many other parts of the body, including the small intestine.
Having a weakened immune system may increase the risk of developing primary CNS lymphoma.
Anything that increases a person's chance of getting a disease is called a risk factor. Not every person with one or more of these risk factors will develop primary CNS lymphoma, and it will develop in people who don't have any known risk factors. Talk with your doctor if you think you may be at risk.
Primary CNS lymphoma may occur in patients who have HIV, AIDS, Epstein-Barr virus, or other disorders of the immune system, or who have had an organ transplant. For more information about lymphoma in patients with AIDS, see AIDS-Related Lymphoma Treatment.
Signs and symptoms of primary CNS lymphoma may include nausea and vomiting or seizures.
These and other signs and symptoms may be caused by primary CNS lymphoma or by other conditions. Check with your doctor if you have any of the following:
- Nausea and vomiting.
- Seizures.
- Headaches.
- Arm or leg weakness.
- Confusion.
- Double vision.
- Hearing loss.
Tests that examine the eyes, brain, and spinal cord are used to diagnose primary CNS lymphoma.
In addition to asking about your personal and family health history and doing a physical exam, your doctor may perform the following tests and procedures:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis depends on the following:
- Whether the patient has HIV.
- The patient's age and general health.
- Whether the tumor is in the central nervous system, eye, or both.
- The level of certain substances in the blood and cerebrospinal fluid (CSF).
Treatment options depend on the following:
- Whether the tumor is in the central nervous system, eye, or both.
- The patient's age and general health.
- Whether the cancer has just been diagnosed or has recurred (come back).
Treatment of primary CNS lymphoma works best when the tumor has not spread outside the cerebrum (the largest part of the brain) and the patient is younger than 60 years, able to carry out most daily activities, and does not have AIDS or other diseases that weaken the immune system.
Staging Primary CNS Lymphoma
After primary central nervous system (CNS) lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the brain and spinal cord or to the eye.
Primary CNS lymphoma usually does not spread beyond the central nervous system or the eye. The process used to find out if cancer has spread is called staging. There is no standard system for staging primary CNS lymphoma.
The following tests and procedures may be used to help plan treatment:
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CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
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PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. A PET scan and CT scan may be done at the same time. This is called a PET-CT.
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MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
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Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.

Bone marrow aspiration and biopsy. After a small area of skin is numbed, a long, hollow needle is inserted through the patient's skin and hip bone into the bone marrow. A sample of bone marrow and a small piece of bone are removed for examination under a microscope.
There is no standard staging system for primary CNS lymphoma.
Primary CNS lymphoma often recurs (comes back) after it has been treated.
Primary CNS lymphoma often recurs in the brain, spinal cord, or the eye.
Treatment of Primary CNS Lymphoma
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed primary central nervous system (CNS) lymphoma may include the following:
- Whole-brain radiation therapy.
- Chemotherapy with or without radiation therapy.
- Targeted therapy alone (rituximab, nivolumab, or ibrutinib).
- Targeted therapy (rituximab) and chemotherapy.
- High-dose chemotherapy and stem cell transplant.
- A clinical trial of high-dose chemotherapy followed by whole-brain radiation therapy, stem cell transplant, and/or targeted therapy.
- A clinical trial of chemotherapy with or without stem cell transplant.
- A clinical trial of targeted therapy (rituximab and ibrutinib) and chemotherapy.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
Treatment of Recurrent Primary CNS Lymphoma
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent primary central nervous system (CNS) lymphoma may include the following:
- Chemotherapy.
- Targeted therapy (rituximab, nivolumab, or ibrutinib).
- Stem cell transplant.
- CAR T-cell therapy.
- Radiation therapy (if not received in earlier treatment).
- A clinical trial of targeted therapy (rituximab and ibrutinib) and chemotherapy.
Treatment of Primary Intraocular Lymphoma
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed primary intraocular lymphoma may include the following:
- Chemotherapy (intraocular or systemic) with or without radiation therapy.
- Whole-brain radiation therapy.
- Chemotherapy with targeted therapy (rituximab).
To Learn More About Primary CNS Lymphoma
For more information from the National Cancer Institute about primary central nervous system (CNS) lymphoma, see the following:
For general cancer information and other resources from the National Cancer Institute, visit:
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PDQ® Adult Treatment Editorial Board. PDQ Primary CNS Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/lymphoma/patient/primary-cns-lymphoma-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389274]
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Last Revised: 2023-05-25
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