Primary CNS Lymphoma - Symptoms, Risk Factors, and Diagnosis

What is Primary CNS Lymphoma and How is it Different than Other Lymphomas?

Lymphoma cancer cell, SEM. Credit: STEVE GSCHMEISSNER/SCIENCE PHOTO LIBRARY

What is primary CNS lymphoma?  What are the symptoms and risk factors, and how is it diagnosed?

Primary CNS Lymphoma - Definition

Primary CNS Lymphoma is a rare form of Non-Hodgkin lymphoma (NHL) that affects only the brain.  This may include the brain itself, the meninges (the membranes which line the brain,) the spinal cord, or the eyes. Other forms of lymphoma may spread to the brain after affecting other parts of the body like lymph nodes.

However, in primary CNS lymphoma no other body part is affected.

Who Gets Primary CNS Lymphoma?

Primary CNS lymphoma is most common in those in their fifties and sixties, though it can occur at any age. It is much more common in those with suppression of the body's immune system, like in AIDS. See the article on Primary CNS Lymphoma and HIV/AIDS if you are coping with primary CNS lymphoma in addition to a diagnosis of AIDS, since the treatments and prognosis are much different. In many parts of the world, the number of patients with Non-Hodgkin lymphoma have increased manifold since the end of the last century, for reasons that are not entirely clear.

Warning Signs

Nearly all people with primary CNS lymphoma have an aggressive form of NHL. The common warning signs include a headache and changes in personality and alertness. Other symptoms may include nausea and vomiting, visual changes, weakness or paralysis, speech changes, or memory loss.

  Symptoms increase fast, and most require seeing a doctor within a few weeks of the start of symptoms.

Tests and Diagnosis

CT and MR scans can often reliably identify a brain tumor as a lymphoma. Brain surgeons need to take a small biopsy from the tumor to confirm it as a lymphoma. This is usually done through a procedure called a stereotactic biopsy.

Complete removal of the tumor is not required. A cerebrospinal fluid (CSF) examination is done by taking a small amount of fluid out of your spinal cord through the back (a spinal tap or lumbar puncture.) Tests are then done to rule out disease in any other part of the body, including a bone marrow biopsy, and likely CT scans of your chest, abdomen, and pelvis. Only then can it be called primary CNS lymphoma.

Treatment of Primary CNS Lymphoma

Treatment of primary CNS lymphoma is different from other types of lymphoma. That is because very few drugs that are useful in other types of lymphoma can reach the brain.  A network of membranes known as the blood brain barrier is meant to protect the brain from toxins, but also "protects" the brain from drugs such as chemotherapy.

A few decades back, radiotherapy to the whole brain was the only effective treatment. Now, chemotherapy with some drugs at high doses is able to take care of the disease better and able to overcome problems faced previously due to lack of penetration of the blood brain barrier.

In addition to chemotherapy and radiation therapy, clinical trials are finding encouraging results with peripheral blood stem cell transplants, as well as targeted therapy (monoclonal antibody therapy in particular.)

See the full article on Primary CNS Lymphoma Treatment to learn about treatment in greater depth.

Outcome and Prognosis

Before the development of effective chemotherapy that can reach the brain, the results of treatment of primary CNS lymphoma were poor. Survival without treatment was on average only 1 to 3 months. Outcomes have improved significantly over the last few decades, and new treatments are being actively studied in clinical trials.  Some patients can now be cured, and many others can now live longer than before.

Support

If you've been diagnosed with this form of lymphoma, don't try to go it alone.  Reach out for help among your family and friends.  You may have a support group in your community, but if you wish to connect with others facing the same disease as you, there is a wonderful lymphoma community available online with many other people coping with primary CNS lymphoma.  Certainly there are side effects of treatment, with some people experiencing  problems with thinking and memory, but there also many ways to work with these symptoms so that you can lead as normal a life as possible.  Take a little time to look into clinical trials, or talk to your doctor about new options available.

Sources:

Batchelor, T. Treatment and prognosis of primary central nervous system lymphoma . UpToDate. Updated 11/15/15. http://www.uptodate.com/contents/treatment-and-prognosis-of-primary-central-nervous-system-lymphoma

National Cancer Institute. Primary CNS Lymphoma Treatment – for health professional (PDQ). Updated 04/02/15. http://www.cancer.gov/types/lymphoma/hp/primary-cns-lymphoma-treatment-pdq#section/_1

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