Lymphomas, also referred to as central nervous system lymphoma or primary central nervous system lymphoma (PCNSL), is a form of Non-Hodgkin's Lymphoma.
What are Lymphomas?
The most common cause of lymphomas is an overproduction of a certain type of white blood cell, called B-lymphocytes. Lymphomas may occur in individuals with healthy immune systems or in patients whose immune system is compromised, such as those who have AIDS or those who have undergone an organ transplant.
Although the incidence of lymphomas is increasing nationwide, these tumors still only account for one to two percent of primary brain tumors. The most commonly affected sites are the cerebral hemispheres but cells can be found in the cerebral spinal fluid (CSF), eyes (ocular), spinal cord, or other organ systems. In patients who do not have an impaired immune system, primary central nervous system tumors have a peak incidence in patients in their 60s and 70s.
Symptoms & Diagnosis
The most common symptom is trouble with thinking, learning or making judgments, called cognitive changes. However, headaches and problems walking, slurred speech or tremor also may occur. When the eyes are affected, it signals more aggressive lymphoma. It may be months or years before the central nervous system is affected.
Diagnosis is made by a biopsy of the affected site after an abnormality has been noted on a MRI of the brain, or through an examination of cells obtained from the cerebral spinal fluid or the vitreous fluid of the eye.
Historically, whole brain radiation has been the primary treatment since lymphoma involves the entire brain. Whole-brain radiation is an effective treatment with approximately 50% of patients showing a complete response. However, when whole brain radiation is not combined with other treatments, patients often relapse. Patients who survive long-term following whole brain radiation often encounter significant cognitive problems related to treatment.
Today, chemotherapy is routinely used alone, or in conjunction with radiation to improve long-term outcomes. The most common chemotherapy drug is methotrexate, which may be administered through intra-venous delivery, intra-arterial delivery or intra-ocular delivery. Chemotherapy also may be given directly into the cerebral spinal fluid through a reservoir (Ommya).
All chemotherapy delivered into the body must cross the blood-brain barrier to obtain entrance into the cerebral spinal fluid, which limits the use of many chemotherapy drugs. Although intra-venous methotrexate with whole brain radiation has been shown to almost double survival, long-term radiation side effects persist.
The Rose Ella Burkhardt Brain Tumor Center is investigating several new treatments currently being used for brain tumor symptoms in clinical trials. Learn more about current clinical trials.