Primary central nervous system lymphoma

Primary central nervous system lymphoma (PCNSL) is an aggressive (fast-growing) type of non-Hodgkin lymphoma (NHL). PCNSL starts in the brain or spinal cord (called the central nervous system, or CNS). It can develop in the membranes that cover and protect the CNS (called the meninges). It can also develop in the eyes (called ocular lymphoma). PCNSL rarely spreads, or metastasizes, outside of the CNS.

Most PCNSLs are B-cell lymphomas, usually diffuse large B-cell lymphoma (DLBCL). But PCNSL can also be a T-cell lymphoma or Burkitt lymphoma.

Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan. When deciding which treatments to offer for PCNSL, they will consider:

• if you have HIV or AIDS
• your age
• your overall health and performance status
• if NHL is in an eye, the CNS or both

When PCNSL is first diagnosed, treatments may include:

• chemotherapy, with or without a stem cell transplant
• radiation to all of the brain (called whole-brain radiation therapy, or WBRT)
• targeted therapy, with or without chemotherapy

HIV- or AIDS-related PCNSL is often treated with highly active antiretroviral therapy (HAART), as well as corticosteroids and whole-brain radiation therapy. Some people may be offered chemotherapy, depending on how well their immune system is working.

People who develop PCNSL after an organ transplant may need to lower the dose of, or stop taking, their immunosuppressant drug.

Steroid therapy for PCNSL uses corticosteroids. A corticosteroid is any steroid hormone that acts as an anti-inflammatory. It reduces swelling in the body.

PCNSL responds very well to corticosteroids. Drugs like dexamethasone or prednisone are used to destroy lymphoma cells and temporarily reduce swelling in the brain tissue.

Chemotherapy uses drugs to destroy cancer cells. To treat PCNSL, chemotherapy may be used alone or with other treatments such as radiation therapy or targeted therapy.

Chemotherapy for PCNSL may be given in different ways. You may have systemic chemotherapy, which means that the drugs travel through the entire body. If lymphoma cells are found in the spinal fluid, the drugs may be given as regional chemotherapy only to the CNS. The drugs are injected directly into the fluid-filled space around the brain and spinal cord. This is called intrathecal chemotherapy.

The most common drug combinations used to treat PCNSL are:

• MATRix – high-dose methotrexate, cytarabine (Cytosar), thiotepa (Tepandia) and rituximab (Rituxan and biosimilars)
• high-dose methotrexate, with or without rituximab
• methotrexate, cytarabine and rituximab
• methotrexate and cytarabine
• thiotepa and busulfan
• ifosfamide (Ifex)
• vincristine
• procarbazine (Matulane)
• temozolomide (Temodal)

Find out more about chemotherapy for NHL.

Targeted therapy uses drugs to target specific molecules (such as proteins) on cancer cells or inside them. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells. Targeted therapy may also be called molecular targeted therapy.

Targeted therapy may be used to treat PCNSL. It may be given with chemotherapy.

The targeted therapy drugs that may be used to treat PCNSL include:

• rituximab
• ibrutinib (Imbruvica)

These targeted therapy drugs may not be covered by all provincial or territorial health plans.

Find out more about targeted therapy for NHL.

Radiation therapy uses high-energy rays or particles to destroy cancer cells. It is sometimes used to treat PCNSL. It may be given after chemotherapy. You may also be offered radiation therapy instead of chemotherapy if you can't have chemotherapy due to other health issues.

Radiation therapy for PCNSL involves giving radiation therapy to all of the brain. This is called whole-brain radiation therapy (WBRT).

Find out more about radiation therapy for NHL.

A stem cell transplant replaces stem cells. It may be offered if PCNSL goes into remission after chemotherapy.

Find out more about stem cell transplant for NHL.

Talk to your doctor about clinical trials open to people with NHL in Canada. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.