What is CNS Lymphoma and How is it Treated?

Medically reviewed by David Reardon, MD

Central nervous system (CNS) lymphoma is an extremely rare form of non-Hodgkin lymphoma that involves the brain and spinal cord, the primary components of your body’s central nervous system.

Non-Hodgkin lymphoma is a type of cancer that originates in lymphocytes, infection-fighting white blood cells that make up your immune system. In CNS lymphoma, cancerous lymphocytes can either amass in the brain and spinal cord (primary CNS lymphoma or PCNSL) or spread to the CNS from other sites of lymph tissue in the body (secondary CNS).

In instances when the lymphoma originates in the eye, known as ocular lymphoma, the disease is still referred to as primary CNS lymphoma because the eye is directly connected to the brain.

What causes CNS lymphoma?

Like many cancers, the direct cause of CNS lymphoma is unknown, but there are some factors – involving a compromised immune system – that may increase your risk. These factors include whether a person has AIDS or another immune system disorder or if the person has undergone medicalimmunosuppression – a therapy used for organ transplants, by which medications “suppress” the immune system to decrease the chance that it will reject the foreign organ.

What are the symptoms of CNS lymphoma?

Similar to other brain or CNS tumors, symptoms of CNS lymphoma may include:

  • Nausea
  • Vomiting
  • Changes in speech or vision
  • Seizures
  • Headaches
  • Confusion
  • Hearing loss
  • Back pain
  • Weakness

Symptoms may also depend on the location of the tumor – for instance, those with ocular lymphoma may experience blurry or double vision, while those whose CNS lymphoma originating in the spinal cord might experience back pain.

Regardless of symptoms – which may or may not present – diagnostic imaging, such as an MRI or PET scan, as well as surgical biopsy or resection, is required to officially diagnose CNS lymphoma.

How is CNS lymphoma treated?

Treatment for primary and secondary CNS lymphoma has evolved over the past few decades, which has overall improved outcomes and reduced the rate of disease recurrence. Broadly, therapies to treat CNS lymphoma may include chemotherapy, radiation, steroids, and stem cell transplant. For some patients, especially those with recurrent CNS lymphoma, participating in a clinical trial may be the best treatment option.

Clinicians and researchers at Dana-Farber’s Center for Neuro-Oncology have shaped treatment options for CNS lymphoma by pioneering a number of clinical trials. Current clinical trials involve treatment options that include immunotherapies and biologically targeted therapies.