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Lymphoma of the brain or spinal cord

This is also known as primary central nervous system (CNS) lymphoma or primary cerebral lymphoma. It is a rare type of lymphoma. Primary means that the lymphoma started in the lymph tissue of the brain or the spinal cord.   

What are lymphomas of the brain or spinal cord?

Lymphoma is a cancer that starts in the lymphatic system. 

The lymphatic system is a system of thin tubes and lymph nodes that run throughout the body. Lymph nodes are bean shaped glands. The thin tubes are called lymph vessels or lymphatic vessels. A fluid called lymph circulates around the body in these vessels and flow through the lymph nodes.

Because the lymphatic system runs throughout your whole body, you can get lymphoma just about anywhere including the brain and the spinal cord. Most lymphomas of the brain start in the front part of the brain (the cerebrum). They can also start in the:

  • spinal cord – lymphoma that starts in the spinal cord is called primary spinal lymphoma
  • eyes – lymphoma that starts in the eyes is called primary intraocular lymphoma
  • layers of tissue that surround the brain (leptomeninges) – lymphoma that starts here is called primary leptomeningeal lymphoma
Diagram showing the main parts of the brain
There are different types of lymphoma. The most common type of primary CNS lymphoma is diffuse large B-cell lymphoma.

Diffuse large B-cell lymphoma develops when the body makes abnormal B cells. These B cells are a type of immune cell that help us to fight infections.

Symptoms of primary CNS lymphoma depend on where the lymphoma started. Symptoms can include:

  • headaches
  • blurred vision
  • changes to your personality
  • seizures (fits)
  • difficulty walking and balancing

How common it is

Lymphoma of the brain or the spinal cord is rare. Around 4 in every 100 brain or spinal cord tumours (4%) are lymphomas. 

What tests will I have?

You have tests to diagnose a lymphoma of the brain or spinal cord. Your doctor checks the size of the tumour and its location. This helps your doctor plan your treatment. The tests you might have include:

  • MRI scan or CT scan
  • a biopsy
  • a test to check for cancer cells in the fluid that surrounds the brain and the spinal cord (a lumbar puncture)
  • blood tests

Treatment

Treatment for lymphoma of the brain is different from other brain tumours. Surgery isn’t usually a possible treatment. This is because it’s difficult to remove these tumours without causing severe side effects.

Chemotherapy is usually the first treatment you have. You have a combination of different chemotherapy drugs that includes the drug methotrexate.

You might have chemotherapy: 

  • as an injection into your bloodstream (intravenously)
  • as an injection into the fluid around the brain and spinal cord (intrathecal chemotherapy)

You have chemotherapy in cycles of treatment. Each cycle usually lasts 3 or 4 weeks. After a number of cycles, you have a scan to see how well the treatment is working.

You might have chemotherapy with a targeted cancer drug called rituximab (Mabthera). Rituximab sticks to a protein called CD20 on the surface of the lymphoma cells. Then the cells of the immune system pick out the marked cells and kills them.

You usually have rituximab as a drip into your bloodstream.

Radiotherapy uses high energy x-rays to destroy cancer cells. You may have radiotherapy: 

  • if you can’t have chemotherapy for any reason 
  • if you have tumour cells left behind after the initial treatment 

You usually have radiotherapy to the whole brain and targeted treatment (boost dose) to the area of lymphoma. 

Follow up

You have regular appointments with your doctor or nurse after treatment finishes. Your doctor examines you at each appointment. They ask how you are feeling, whether you have had any symptoms or side effects, and if you are worried about anything. You also have MRI scans on some visits.

How often you have check ups depends on your individual situation.

For a lymphoma of the brain or the spinal cord, you usually have an MRI scan every 3 to 4 months, for up to 2 years.

Coping with lymphoma of the brain or spinal cord

Coping with a diagnosis of cancer can be difficult, both practically and emotionally. It can be especially difficult when you have a rare cancer. Being well informed about the type of cancer you have, and its treatment can make it easier to cope.  

Research and clinical trials

Doctors are always trying to improve the diagnosis and treatment of brain tumours. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or look at different combinations of existing treatments.

Last reviewed: 
30 Oct 2019
  • Guidelines for the diagnosis and management of primary central nervous system diffuse large B-cell lymphoma
    C Fox and others
    British Journal of Haematology, 2018. Vol. 184, issue 3

  • Primary CNS Lymphoma
    Christian Grommes and Lisa DeAngelis
    Journal of Clinical Oncology, 2017. Vol 35, Number 21, Pages 2410-2418

  • Cancer: Principles & Practice of Oncology (10th edition)
    VT DeVita, TS Lawrence and SA Rosenberg
    Lippincott, Williams and Wilkins, 2015

  • The 2016 World Health Organisation Classification of Tumors of the Central Nervous System: a summary
    DN Louis and others
    Acta Neuropathologica, 2016. Vol 131, Issue 6, Pages 803-820

  • Clinical presentation, pathologic features, and diagnosis of primary central nervous system lymphoma
    Tracey Batchelor and Jay S Loeffler
    UpToDate, Last August 2019

  • Treatment and prognosis of primary central nervous system lymphoma
    Tracer Batchelor
    UpToDate, Last accessed August 2019

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