Primary Central Nervous System Lymphoma

Primary central nervous system lymphoma (CNS) is an aggressive form of NHL in which malignant (cancer) cells form in the lymph tissue of the brain and/or spinal cord. It may develop in the brain, spinal cord, eye, or leptomeninges (two of the membranes that surround the brain and spinal cord).

When lymphoma has originated in other parts of the body and subsequently has spread to the CNS, it is referred to as secondary CNS lymphoma. The main symptoms of CNS lymphoma are focal neurological deficits (i.e., problems with nerve, spinal cord, or brain function), but headaches, vomiting, confusion, seizures, personality changes, and blurred vision can also occur.

The cause of CNS lymphoma is unknown, but there are some factors that may increase the risk of developing it, such as infection with Epstein-Barr virus (EBV) or human immunodeficiency virus (HIV); a compromised immune system (which may be the case for people with acquired immunodeficiency syndrome [AIDS] or patients who have undergone organ transplant); exposure to chemicals such as pesticides, solvents, or fertilizers; and a family history of NHL.

Having one or more of these risk factors does not mean a person will develop NHL. Most people with risk factors never develop the disease and many people diagnosed have never been exposed to any clearly identifiable risk factors.

To learn more about primary central nervous system lymphoma, download the Central Nervous System Lymphoma Fact Sheet.