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Primary Brain Lymphoma (Primary CNS Lymphoma)

Cancer that forms in the lymph tissue of the brain, spinal cord, meninges (outer covering of the brain), or eye (called ocular lymphoma).

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

About Primary Brain Lymphoma (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).

Having a weakened immune system may increase the risk of developing primary CNS lymphoma.

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean... Read more about Primary Brain Lymphoma

What works? Research summarized

Evidence reviews

The role of brain radiotherapy (X‐rays) in the treatment of lymphoma in the brain

Background: Primary central nervous system lymphoma (PCNSL) is a type of cancer that occurs in the brain or spinal cord. It is a rare and aggressive type of lymphoma. People who develop PCNSL survive for only four months on average, if they do not receive treatment. For a long time the only treatment showing any benefit was whole brain radiotherapy (WBR), in which X‐rays are used to destroy cancerous cells in the brain. However, several studies suggest that this treatment method also produces signs of damage to healthy brain tissue. Since the introduction of methotrexate, a powerful chemotherapy drug showing great beneficial effects, experts have debated the role of radiotherapy in the treatment of people with PCNSL. Radiotherapy could be combined with chemotherapy, or not used at all, especially considering its potentially harmful effects.

Non-Hodgkin's Lymphoma: Diagnosis and Management

Non-Hodgkin's lymphoma is the sixth most common cancer in the UK. There are many different subtypes of the disease, with markedly different clinical courses and requirements for therapy. Diagnosing non-Hodgkin's lymphoma and the precise subtype is challenging, and optimising the diagnostic process is central to improved management. Significant improvements in our understanding of the biology of non-Hodgkin's lymphoma have contributed to improved diagnosis and also allowed for more targeted therapies.

Quality of haematological and oncological care in children: Executive summary of final report V06-01, Version 1.0

The aim of the investigation was to evaluate the quality of current medical care in Germany for children with haemato-oncological diseases.

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Summaries for consumers

The role of brain radiotherapy (X‐rays) in the treatment of lymphoma in the brain

Background: Primary central nervous system lymphoma (PCNSL) is a type of cancer that occurs in the brain or spinal cord. It is a rare and aggressive type of lymphoma. People who develop PCNSL survive for only four months on average, if they do not receive treatment. For a long time the only treatment showing any benefit was whole brain radiotherapy (WBR), in which X‐rays are used to destroy cancerous cells in the brain. However, several studies suggest that this treatment method also produces signs of damage to healthy brain tissue. Since the introduction of methotrexate, a powerful chemotherapy drug showing great beneficial effects, experts have debated the role of radiotherapy in the treatment of people with PCNSL. Radiotherapy could be combined with chemotherapy, or not used at all, especially considering its potentially harmful effects.

Primary CNS Lymphoma Treatment (PDQ®): Patient Version

Expert-reviewed information summary about the treatment of primary CNS lymphoma.

Childhood Hodgkin Lymphoma Treatment (PDQ®): Patient Version

Expert-reviewed information summary about the treatment of childhood Hodgkin lymphoma.

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Terms to know

Brain
The part of the central nervous system that is contained within the skull (cranium).
Central Nervous System
The brain and spinal cord. Also called CNS.
Eye
Eyes are the organs of vision. They detect light and convert it.
Immune System
The body's system for protecting itself from viruses and bacteria or any foreign substances.
Intraocular
Within the eyeball.
Lymph
The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.
Lymphatic System
The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.
Lymphocytes
A lymphocyte is a type of white blood cell that is part of the immune system. There are two main types of lymphocytes: B cells and T cells. The B cells produce antibodies that are used to attack invading bacteria, viruses, and toxins. The T cells destroy the body's own cells that have themselves been taken over by viruses or become cancerous.
Lymphoma
Cancer that begins in cells of the immune system.
Meninges
The three thin layers of tissue that cover and protect the brain and spinal cord.
Spinal Cord
A column of nerve tissue that runs from the base of the skull down the back. It is surrounded by three protective membranes, and is enclosed within the vertebrae (back bones). The spinal cord and the brain make up the central nervous system, and spinal cord nerves carry most messages between the brain and the rest of the body.
Tissue
A group of cells that act together to carry out a specific function in the body. Examples include muscle tissue, nervous system tissue (including the brain, spinal cord, and nerves), and connective tissue (including ligaments, tendons, bones, and fat). Organs are made up of tissues.

More about Primary Brain Lymphoma

Photo of an adult

Also called: Microglioma, Primary central nervous system lymphoma, PCNSL

See Also: Intraocular Lymphoma

Other terms to know: See all 12
Brain, Central Nervous System, Eye

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