Brain Tumor Program: Primary Central Nervous System (CNS) Lymphoma

Primary Central Nervous System Lymphomas: specialized care

Primary central nervous system (CNS) lymphoma is a cancerous (malignant) tumor affecting white blood cells (lymphocytes) in the brain or spinal cord. Lymphocytes play an important role in helping your immune system fight off germs and infection.

Primary CNS lymphoma is a rare tumor and has a tendency to come back, which can make it difficult to treat. Our team of experts offers a unique depth of expertise in researching, diagnosing and treating patients with primary CNS lymphoma.

UCI Health brain tumor specialists are the region’s experts in primary central nervous system (CNS) lymphoma treatment. We offer advanced treatments, such as chemotherapy, clinical trials and TrueBeam™ radiation therapy.

Our integrated care approach means that many specialists are involved in determining the most effective treatment plan for you. Together, our goal is to provide you with the most seamless, effective care possible. Learn more about radiation therapy ›

Highlights of our program include:

• Expertise: Even though primary CNS lymphomas are rare, our depth of experience in treating this condition translates to a superior level of care. 
• Collaborative care: Our team includes experts in blood cancers (hematology), brain tumors (neuro-oncology), radiation oncology, neurosurgery and neuro-ophthalmology. Working side by side, we deliver seamless treatments to help you achieve the best possible outcomes while avoiding potential complications. Meet our team ›
• Clinical trials: Our brain tumor experts are exploring new treatment options for primary CNS lymphoma, such as new medication options for patients whose tumors return after treatment (refractory primary CNS lymphoma). Learn more about research and clinical trials ›
• Patient support: Whether you need emotional support or extra help at home, we can help make your life a little less stressful during treatment. Learn more about our patient support services ›

What is primary central nervous system lymphoma?

Primary CNS lymphoma can affect any part of the nervous system or surrounding organs, including the brain, spinal cord, spinal fluid (which cushions the brain and spinal cord) and eyes. This condition gets its name from the fact that these tumors form in the central nervous system and nowhere else in the body.

Everyone has a different experience with primary CNS lymphoma, meaning some or all parts of the nervous system can be affected. Primary CNS lymphomas rarely travel (metastasize) outside of the nervous system.

Symptoms of primary central nervous system lymphoma

The most common symptom of primary CNS lymphoma is a headache. As the tumor grows, it quickly takes up all the extra space within the skull, putting pressure on the brain and causing serious headaches. Headaches may also stem from blockages in the fluid-filled sacs of the brain that leads to a build-up of spinal fluid, which also puts pressure on the brain.  

Symptoms may include:

• Nausea and vomiting
• Bursts of abnormal electrical activity in the brain (seizures)
• Having trouble thinking, becoming confused easily
• Changes in behavior and personality
• Muscle weakness in the arms and legs
• Changes in vision, including double vision
• Hearing loss

Causes of primary central nervous system lymphoma

Patients with weakened immune systems, including patients who have had an organ transplant or have acquired immunodeficiency syndrome (AIDS), face a higher than normal risk of developing primary CNS lymphoma. Otherwise, researchers are not exactly sure what causes this type of tumor.

Diagnosing primary central nervous system lymphoma

We have a high level of diagnostic expertise, ensuring that patients receive an accurate diagnosis. Together with our brain tumor specialists, our imaging and lab specialists analyze diagnostic test results to create an effective treatment plan.

Tests we use to diagnose primary CNS lymphoma include:

• Neurologic exam: During a neurologic exam, we ask questions and have you perform simple tasks to help us assess your vision, coordination and balance, as well as to detect changes in mood or behavior.
• Lumbar puncture: During a lumbar puncture, we insert a hollow needle into the lower part of your back (lumbar section) and take a sample of spinal fluid. Also known as a spinal tap, this test is used to look for signs of infection or rule out other medical conditions. Learn more about getting a lumbar puncture ›
• Eye exam: It is possible for a primary CNS lymphoma to affect your vision, which is why you may receive an eye examination. Learn more about eye care services ›
• Imaging tests: Imaging tests, such as a computed tomography (CT) scan, can help us determine the precise size and location of the tumor.

• Biopsy: We use this test to gather a tissue sample (biopsy) from the brain or spinal cord, which can help us confirm or rule out a primary CNS lymphoma diagnosis. A brain biopsy requires a surgical procedure, but you can breathe a little easier knowing our neurosurgeons are among the best in the country. Learn more about neurosurgery ›

Primary central nervous system lymphoma treatments

Your treatment plan depends on the size and location of the tumor, the affected organs and your overall health. Our personalized approach means we only recommend treatments that effectively destroy the tumor and are responsive to your individual health needs.

Treatments for primary CNS lymphoma include:

• Steroids: Steroids are medications we use to temporarily shrink the tumor, which can relieve symptoms associated with swelling in the brain.
• Targeted therapy: This treatment is a form of chemotherapy and uses special medications to strengthen or attack specific cells and functions. Targeted therapy for primary CNS lymphoma works to slow (inhibit) lymphoma cell growth, causing the tumor to shrink.
• Radiation: Radiation therapy uses powerful, high-energy X-ray beams to shrink and destroy tumors. We are among the first programs in Orange County to offer the latest radiation therapy technologies, including the TrueBeam™ system. Learn more about the TrueBeam Radiotherapy and Radiosurgery System › 

Contact us

To schedule an appointment, call 714-456-8000 or fill out an online request form. You may also send us an email.

Our 24-hour promise: If you are a new patient, one of our brain tumor physicians will return your call within 24 hours. You can be seen in our offices within 48 hours after insurance approval.