Central nervous system (CNS) lymphoma is a condition in which malignant cells are present in the lymph tissue of the brain and/or spinal cord. Treatment for CNS lymphoma generally does not include surgery. Instead, nonsurgical treatments, namely corticosteroids, chemotherapy and/or whole-brain radiation therapy, are used to treat CNS lymphoma.
Lymphoma designates a group of cancers that arise from white blood cells called lymphocytes. Because lymphocytes travel in the blood and lymph tissue and pass in and out of the CNS, lymphoma too can develop in various places in the body, including the CNS. CNS lymphoma is an aggressive type of lymphoma.
CNS lymphoma comes in two forms, depending on the origin of the tumor, and can produce single or multiple growths.
- Primary CNS lymphoma originates in the lymph tissue of the brain or spinal cord.
- Secondary CNS lymphoma originates in lymph tissue outside of the CNS and then metastasizes to the CNS.
Primary and secondary CNS lymphoma are both very rare, with primary CNS lymphoma making up only about 2 to 3 percent of primary brain tumors.
CNS lymphoma can be found in the cerebrum, leptomeninges, eyes and, very rarely, the spinal cord.
Treatment for CNS lymphoma does not aim to cure the disease—unfortunately, no available treatments can cure the disease. Instead, the goals of treatment are to extend life and improve quality of life.
Symptoms depend on the location of the tumor or tumors. Some symptoms include headache, nausea and vomiting, seizures, changes in behavior, ataxia, hearing loss, dysphasia, hemiparesis, and aphasia. If the eye is involved, visual disturbances, such as blurred vision, may occur. Tumors present in the spinal cord may lead to back pain, incontinence, or leg weakness.
Usually, a neurological examination is conducted to assess symptoms. This exam consists of evaluating swallowing, hearing, sense of smell, eye movements, sensation, motor function, balance, and coordination. Also, an eye exam may be performed to evaluate eye health and determine any problems.
Imaging studies are the primary component of diagnosis. Computed tomography (CT) or magnetic resonance imaging (MRI) scans can be used to image the brain. Either scan may be performed with or without contrast enhancement. Typically, an MRI is conducted on the brain and the spine to identify all possible tumors in the CNS, and a CT scan is performed on other areas of the body to identify tumors outside the CNS. A chest X-ray may be done to check for tumors in the chest.
In addition, blood tests may be ordered to obtain a complete blood count and test for human immunodeficiency virus (HIV).
Biopsy of the tumor is typically required to confirm a diagnosis for primary CNS lymphoma (and may or may not be necessary for secondary CNS lymphoma). Usually, a stereotactic or open biopsy is performed to acquire a tumor tissue sample. However, other methods can be used in some cases: A lumbar puncture may be used to obtain cancer cells from the cerebrospinal fluid, or a vitrectomy may be performed to determine whether cancer cells are in the eyes.
What causes CNS lymphoma is currently unclear. Most instances of CNS lymphoma are sporadic.
Individuals who are immunocompromised have a heightened risk of developing CNS lymphoma. Such individuals most often also have human immunodeficiency virus (HIV) or acquired immunodeficiency syndrome (AIDS). People with other immune deficiency conditions, such as rheumatoid arthritis, telangiectasia and Wiskott-Aldrich syndrome, are also at elevated risk, as are those who have had an organ transplant.
About twice as many men as women have CNS lymphoma. CNS lymphoma tends to occur among individuals in their 50s to 70s, but for people with HIV/AIDS, the average age is much lower—in the mid 30s.
At Columbia, our neurosurgeons use the latest treatments and most sophisticated techniques to treat CNS lymphoma, providing patients with the best possible results.
CNS lymphoma is typically not treated by surgical resection because this type of tumor tends to have poorly defined borders, making complete resection difficult. Since the disease is present in, and travels through, the lymph system, complete or even partial resection of a lesion does not improve overall prognosis. Very rarely, partial tumor resection may be performed in order to relieve high intracranial pressure and accompanying symptoms, but this procedure typically does not improve overall disease prognosis.
Primary treatment for CNS lymphoma may include more than one kind of nonsurgical therapy. The plan of management for an individual patient depends on a number of variables, such as the patient’s overall health. Our neurosurgeons and team of expert specialists determine the course of treatment that is best for each patient’s condition.
- Corticosteroids are often used to shrink a tumor quickly and provide immediate symptom relief, but additional treatment is required.
- Chemotherapy can be a very effective treatment option and is used either alone or in combination with whole-brain radiation therapy.
- Whole-brain radiation therapy can be administered to destroy single or multiple tumors in the brain. However, this option is typically avoided in patients older than 60 because the treatment can negatively affect brain function, including memory. Whole-brain radiation therapy used to be the only treatment available, but due to medical advances, chemotherapy may now take its place for many patients.
A patient’s prognosis depends on a variety of factors, such as age and whether the patient is immunocompromised. Although treatment cannot cure this aggressive disease, it can improve a patient’s quality of life and extend time with loved ones.