What is primary CNS lymphoma?
Primary CNS lymphoma (PCNSL) means lymphoma that starts in the brain, spinal cord or eye. Together these areas are called the central nervous system (CNS).
Lymphoma is a type of blood cancer. It develops when blood cells called lymphocytes become abnormal. These blood cells normally help fight infection.
Lymphoma that starts in the CNS is rare. It more commonly starts in other areas of the body. There are different subtypes of lymphoma. The most common type of PCNSL is a fast-growing non-Hodgkin lymphoma called diffuse large B-cell lymphoma (DLBCL).
PCNSL may affect one or several areas of the brain or spinal cord. If it affects the eye, it is called intraocular lymphoma or vitreoretinal lymphoma.
What is secondary CNS lymphoma?
Secondary CNS lymphoma is lymphoma that has started in another part of the body but:
- is in the brain at diagnosis
- spreads to the brain later.
It may be treated differently from primary CNS lymphoma.
Related pages
Booklets and resources
Symptoms of primary CNS lymphoma
Symptoms of primary CNS lymphoma may depend on:
- where the tumour is in the brain or spinal cord
- how quickly it grows.
Possible symptoms can include:
- behaviour or personality changes – these are common, but changes are often minor to start with, and may only be noticed by close family, friends or carers
- problems with memory, concentration and making decisions
- numbness, tingling or weakness – this may affect one side of the body or the arm or leg
- speech problems – this may include difficulties with finding words or forming sentences
- changes in balance or movement – this may affect co-ordination and walking
- fits (seizures)
- problems with eyesight, such as blurred vision or blind spots – these can happen if the lymphoma starts in or near the eye or in the part of the brain that controls vision
- headaches and vomiting – the lymphoma may cause swelling or blockages which increase pressure in the skull. Headaches may be worse in the morning or get worse when you cough, sneeze or bend down.
Worried about symptoms?
A change in how you feel or how your body works can be caused by conditions other than PCNSL. But always ask your GP to check any ongoing, new or unexplained symptoms.
We are also here if you need someone to talk to. You can:
- call the Macmillan Support Line free on 0808 808 00 00
- chat to our specialists online.
Related pages
Causes of primary CNS lymphoma
It is not clear why lymphoma starts in the central nervous system. We have more general information about the causes and risk factors of non-Hodgkin lymphoma.
For some people, PCNSL may be linked to immunosuppression. This means having a weakened immune system. A weakened immune system may be caused by:
- conditions such as HIV
- drugs called immunosuppressants – these are used after an organ transplant, or to treat autoimmune disease.
Like other cancers, PCNSL is not infectious. You cannot spread it to other people.
Diagnosis of PCNSL
If you have symptoms that worry you, you should begin by going to your GP. They will examine you. If they think cancer could be causing your symptoms, they will refer you to a specialist doctor at a hospital. The most common tests for diagnosing PCNSL are:
A biopsy is when a surgeon removes a piece of the tumour from the affected area. This is sent to a laboratory for testing.
There are different ways to take a brain biopsy. Your surgeon can explain more about biopsies.
Further tests for PCNSL
You may also have tests to check for signs of lymphoma in other parts of your body. These may include:
- blood tests
- a CT scan
- a PET-CT scan
- a lumbar puncture
- a bone marrow biopsy
- eye tests
- an ultrasound scan of the testicles.
Your doctor will usually ask you whether they can take a blood sample to check for HIV and other infections such as hepatitis B and C. They can plan safer and more effective treatment for you if they know you have these infections.
Waiting for test results
Waiting for test results can be a difficult time. It may take up to 2 weeks for the results to be ready.
You may find it helpful to talk to family members or a close friend. Your specialist nurse can also provide support.
You can talk to one of our cancer support specialists for free on 0808 808 0000, 7 days a week, 8am to 8pm.
Related pages
Booklets and resources
Driving and PCNSL
After diagnosis and treatment for PCNSL, you will not be allowed to drive for a period of time.
If you do drive, this change may be upsetting and frustrating. But it is a legal requirement. It is important to follow the advice you are given.
If you live in England, Scotland or Wales, you must contact the Driver and Vehicle Licensing Agency (DVLA) to tell them about your illness. If you live in Northern Ireland, contact the Driver and Vehicle Agency (DVA).
Treatment for primary CNS lymphoma
A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).
After the MDT meeting, you will usually meet with your cancer doctor and specialist nurse. They will explain the different treatments and their side effects. They will also talk to you about things to consider when making treatment decisions.
You usually have treatment for PCNSL in 2 phases:
- First phase – this is usually chemotherapy. The aim is to make as much of the lymphoma disappear as possible. This is called remission.
- Second phase – this is to treat any remaining lymphoma cells. The treatment will try to prevent PCNSL from coming back in the future (relapse). This phase is called consolidation. Consolidation treatment can include high dose chemotherapy with stem cell transplant, and radiotherapy.
You may have some treatments as part of a clinical trial.
Treatment for PCNSL may include:
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Immunotherapy and chemotherapy
Often the first treatment for PCNSL is a combination of the immunotherapy drug rituximab and chemotherapy. This is called chemoimmunotherapy. Combinations used for PCNSL include:
- MATRix – this is made up of rituximab and the chemotherapy drugs methotrexate, cytarabine and thiotepa. This is an intensive treatment. Intensive treatment can cause more severe side effects and will not be suitable for everyone. If you have other health conditions, your cancer team will plan a less-intensive combination of cancer drugs for you.
- RMA – this is made up of rituximaband the chemotherapy drugs methotrexate and cytarabine. This is also an intensive treatment.
- Methotrexate and rituximab with either temozolomide or procarbazine.
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Radiotherapy
Radiotherapy uses high-energy rays called radiation to treat cancer. It destroys cancer cells in the area you have it.
You might have radiotherapy after chemotherapy. Or you may have radiotherapy on its own if you are not well enough to have chemotherapy. Radiotherapy is usually used to treat the whole brain. When lymphoma starts in the eye, radiotherapy can sometimes be given directly to the eye.
Your cancer team will explain possible side effects of your treatment. For radiotherapy to the whole brain, the risk of side effects may depend on several factors, including the dose of treatment you have. Some treatments can cause long term changes such as problems with memory, thinking or reasoning.
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High dose chemotherapy with stem cell transplant
This is sometimes used to treat PCNSL. It is an intensive treatment, so it is not suitable for everyone. You have intensive chemotherapy treatment then a transplant using your own stem cells (autologous stem cell transplant). The stem cells help your body recover from the effects of the chemotherapy.
PCNSL can respond well to treatment. Having intensive treatment may cure PCNSL. But, for some people, the lymphoma comes back. This is called relapse or recurrence. If this happens, the lymphoma cannot usually be cured. It may be possible to have more treatment to control the lymphoma and manage any symptoms.
Treating symptoms of primary CNS lymphoma
PCNSL can increase the pressure in the skull or spine. For some people, this can cause symptoms that need treatment straight away. You may be given drugs called steroids. These may shrink the lymphoma for a time. Steroids can also be used to reduce any swelling around the lymphoma.
You usually start taking steroids after you have had a biopsy. Often you will only take them for a few weeks, and you may be able to stop soon after you start chemotherapy.
Rarely, surgery may be used to reduce the pressure.
If you have seizures caused by PCNSL, you may be given drugs called anticonvulsants to help prevent them.
After primary CNS lymphoma treatment
Follow-up after treatment for PCNSL
You will have regular check-ups during and after your treatment for PCNSL. Your cancer doctor or specialist nurse will explain what to expect.
Follow-up appointments are a good time for you to talk to your cancer doctor or specialist nurse about any concerns you have. Tell them as soon as possible if you have any problems or notice new symptoms between appointments. We have more information about follow-up care after treatment.
Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about late effects of lymphoma treatment.
Wellbeing and recovery
Looking after yourself can help speed up your recovery after PCNSL treatment. Even small lifestyle changes may improve your wellbeing and long term health.
Even if you already have a healthy lifestyle, you may choose to make some positive changes after treatment. We have more information on leading a healthy lifestyle after treatment.
Support for people with PCNSL
It is important to know where to get support or information if you need it. People often need support even some time after MCL treatment. But sometimes it is difficult to know who to ask for help.
You can:
- ask your GP or someone from your cancer team for advice about support in your area
- find cancer support services near you
- call the Macmillan Support Line for free on 0808 808 00 00.
- chat to our cancer information specialists online.
- visit our non-Hodgkin lymphoma forum to talk to people who have been affected by lymphoma, share your experience, and ask your questions.
You may also find the HOPE programme helpful. The programme is a free 6 week self-management course designed to help you develop techniques and strategies when living with or after cancer. Topics include goal setting, fatigue management, and wellbeing.
You can sign up for the online HOPE programme. Or to find out more about face to face programmes in your area, email ServiceOpsSupport@macmillan.org.uk
Other organisations who offer information and support
- Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.
- Lymphoma Action provides high quality information and support for people affected by lymphoma. It provides helpline services and a range of peer support including online support meetings, educational events and webinars. Its website includes TrialsLink, a database of lymphoma clinical trials.
More information and advice
We know cancer can affect you physically, emotionally and financially. We have information and advice about different ways cancer might impact you, such as help with:
About our information
This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer.
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References
Below is a sample of the sources used in our lymphoma information. If you would like more information about the sources we use, please contact us at informationproductionteam@macmillan.org.uk
Cwynarski K, Marzolini MAV, Barrington SF, et al. The management of primary mediastinal B-cell lymphoma: a British Society for Haematology Good Practice Paper. Br J Haematol. 2019;185:402-9.
Eyre TA, Bishton MJ, McCulloch R, et al. Diagnosis and management of mantle cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(1):108–26.
Ferreri AJM, Illerhaus G, Doorduijn K, et al., on behalf of the EHA and ESMO Guidelines Committees. Primary central nervous system lymphomas: EHA–ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2024;35(6):491-507.
Fox CP, Ahearne MJ, Pettengell R, et al. Guidelines for the management of mature T- and natural killer-cell lymphomas (excluding cutaneous T-cell lymphoma): a British Society for Haematology Guideline. Br J Haematol. 2022;196:507-22.
Fox CP, Chaganti S, McIlroy G, et al. The management of newly diagnosed large B-cell lymphoma: A British Society for Haematology Guideline. Br J Haematol. 2024;204(4):1178–92.
McNamara C, Montoto S, Eyre TA, et al. The investigation and management of follicular lymphoma. Br J Haematol. 2020;191:363-81.
Pratt G, El-Sharkawi D, Kothari J, et al. Diagnosis and management of Waldenström macroglobulinaemia—A British Society for Haematology guideline. Br J Haematol. 2022;197:171–87.
Walewska R, Eyre TA, Barrington S, et al. Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline. Br J Haematol. 2024;204(1):86–107.
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