Primary CNS lymphoma (PCNSL) is a rare type of lymphoma which starts in the central nervous system (CNS).
PCNSL may affect one or several areas of the brain or spinal cord. It may also affect the eye. This is called intraocular lymphoma or vitreoretinal lymphoma. PCNSL does not affect other areas of the body, such as the lymph nodes.
PCNSL develops when the body makes abnormal (cancerous) lymphocytes. Lymphocyte are white blood cells that normally help fight infection. PCNSL usually develops from B-cell lymphocytes. The most common type is a fast-growing non-Hodgkin lymphoma called diffuse large B-cell lymphoma (DLBCL).
A lymphoma that starts in another part of the body but is also in the brain at diagnosis, or spreads there later is called a secondary CNS lymphoma. It may be treated differently to primary CNS lymphoma.
Primary CNS lymphoma (PCNSL) may cause different symptoms, depending 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 in primary CNS lymphoma, but changes are often slight to start with and may only be noticed by close family or friends.
- Finding it difficult to remember things. Problems with concentration or making decisions.
- Numbness, tingling or weakness. This may include numbness or weakness on one side of the body, or tingling and weakness in an arm or leg.
- Speech problems – difficulties with finding words or forming sentences.
- Changes in balance or movement. If your balance and co-ordination are affected, walking may be more difficult.
- 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 your vision.
- Headaches and vomiting. Sometimes the lymphoma can increase the pressure in the skull. This may happen because of swelling or blockages caused by the tumour.
We have more information about these and other symptoms of brain tumours.
The causes of primary CNS lymphoma (PCNSL) are mostly unknown.
There are certain things that can affect the chances of developing PCNSL. These are called risk factors. Your risk of developing PCNSL may be higher if your immune system is weak. Your immune system can be weakened by:
- conditions such as HIV
- drugs called immunosuppressants – these are used after an organ transplant, or to treat autoimmune disease.
But most people diagnosed with PCNSL do not have immune system problems.
Like other cancers, PCNSL is not infectious. It cannot be passed on to other people.
The most common tests for diagnosing primary CNS lymphoma (PCNSL) are:
A biopsy is when the surgeon removes a piece of the tumour from the affected area. The tissue will be sent to a laboratory for testing.
There are different ways to take a brain biopsy. Your surgeon can explain more about biopsies.
We have more information about diagnosing brain tumours.
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
- bone marrow samples
- eye tests
- an ultrasound scan of the testicles.
Your doctor will usually ask you if 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 when they know if you have these infections.
Driving and PCNSL
You will not be allowed to drive from the time of your diagnosis until at least 2 years after your treatment has ended.
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).
A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).
This will usually include:
- a lymphoma doctor (an oncologist or haematologist)
- doctors who specialise in treating brain conditions (neurologists or neurosurgeons)
- specialist nurses.
Your doctor, cancer specialist or nurse will explain the different treatments and their side effects to you. They will also talk to you about things to consider when making treatment decisions.
Treatment for primary CNS lymphoma (PCNSL) is usually given in 2 stages:
- First stage – this is normally chemotherapy. The aim is to get rid of all of the lymphoma. This is called remission.
- Second stage – this is to treat any remaining lymphoma cells that can’t be seen on tests. The treatment will try to prevent the PCNSL from coming back in the future (a relapse). This stage is called consolidation. Consolidation treatment can be with high-dose chemotherapy, a stem cell transplant, radiotherapy or chemotherapy tablets.
Treatments for PCNSL may include:
Immunotherapy and chemotherapy
Often the first treatment for PCNSL is a combination of the immunotherapy drug rituximab and chemotherapy. This is called chemoimmunotherapy. A treatment called MATRix 4 is a common combination of drugs for PCNSL. This is made up of rituximab and the chemotherapy drugs methotrexate, cytarabine and thiotepa. Another combination is methotrexate and rituximab with either temozolomide or procarbazine.
Radiotherapy uses high-energy rays to destroy cancer cells Radiotherapy may be given after chemotherapy, or on its own if you are not well enough to have chemotherapy. For PCNSL, radiotherapy is usually used to treat the whole brain. When lymphoma starts in the eye, radiotherapy can sometimes be given directly to the eye.
Stem cell transplant
You may have some treatments as part of a clinical trial.
Treating symptoms of PCNSL
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.
You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you may have at these appointments. Your doctor will want to know how you are feeling, and to check you are recovering from any side effects of treatment.
Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about long-term and late effects of treatment for lymphoma.
Sex life and fertility
Cancer and its treatment can sometimes affect your sex life. There ways to improve your sexual well-being and to manage any problems.
Treatment for lymphoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:
Recovering after treatment for a brain tumour
It may take some time before your symptoms improve after treatment for a brain tumour. You may have ongoing side effects or have to adjust to some physical or emotional changes caused by the brain tumour. Your healthcare team includes different professionals who can help as you recover.
Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.
Macmillan is also here to support you. If you would like to talk, you can:
The organisations below also offer information and support:
Blood Cancer UK
Lymphoma Action gives emotional support, advice and information for people with Hodgkin lymphoma or non-Hodgkin lymphoma and those close to them. It has a national network of people with lymphoma, as well as local groups. Their website has a section called trialslink where you can see information about lymphoma clinical trials.
The Brain Tumour Charity
Below is a sample of the sources used in our primary CNS lymphoma (PCNSL) information. If you would like more information about the sources we use, please contact us at firstname.lastname@example.org
Non-Hodgkin’s lymphoma: diagnosis and management; NICE Guideline (July 2016).
British Society for Haematology. Guidelines for the diagnosis and management of primary central nervous system diffuse large B-cell lymphoma. 2018. British Journal of Haematology, 2019, 184, 348–363.
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. It has been approved by Senior Medical Editor, Dr Anne Parker, Consultant Haematologist.
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