Lymphoma is a cancer that arises from the cells of the lymphatic system. In the brain, this type of cancer is called Primary CNS Lymphoma (PCNSL).
Lymphoma occurs most often in the cerebral hemisphere, but may also involve the cerebrospinal fluid, the eyes, or the spinal cord. In addition, some people may have evidence of lymphoma elsewhere in the body. It is not unusual for this tumor to be found in multiple areas of the cerebral hemisphere, as it can spread throughout the central nervous system.
The most common symptoms of CNS lymphoma include personality and behavioral changes, confusion, symptoms associated with increased pressure within the brain (eg, headache, nausea, vomiting, drowsiness), weakness on one side of the body, and seizures. Problems with eyesight may also occur.
Once a diagnosis is confirmed, steroids are used to control brain swelling; this may result in the immediate disappearance of the tumor on a later scan. Chemotherapy and radiation, or chemotherapy alone may then be used the primary treatment. Surgery is not usually an option because lymphomas tend to occur deep within the brain and the risk of surgical complications is too high.
This disease affects people with healthy immune systems, as well as those whose immune systems are not functioning properly, for example organ transplant recipients, patients with autoimmune disease or people who are HIV positive.
The incidence of CNS lymphoma has been increasing over the past 20 years; it now represents between 2% and 3% of all primary brain tumors.