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Central nervous system (CNS) lymphoma is a cancer that affects the lymphatic system in the brain or spinal cord. It begins in immune cells called lymphocytes and weakens the body’s defenses against pathogens. CNS lymphoma is a rare form of non-Hodgkin lymphoma. Non-Hodgkin lymphoma is a type of cancer that affects the lymph system, a part of the immune system. In CNS lymphoma, cancer cells grow in the brain or spinal cord. This article looks at CNS lymphoma in more detail, including the causes and symptoms. It then discusses how doctors diagnose and treat the disease. Share on PinterestRafe Swan/Getty Images CNS lymphoma is a type of non-Hodgkin lymphoma. In this condition, cancer cells form in the lymph tissue in the brain or spinal cord. Secondary CNS lymphoma can also happen if cancer cells from other parts of the body spread to the brain or spinal cord. Non-Hodgkin lymphoma develops in white blood cells called lymphocytes. In about 95% of cases, CNS lymphoma affects the B lymphocytes, which are cells that make antibodies. In the remaining cases, CNS lymphoma affects T lymphocytes. These cells help fight infection by activating other cells, destroying germs, or slowing the growth of dangerous invasive pathogens. CNS lymphoma is more aggressive than other forms of lymphoma, with a 5-year survival rate of 30%. In people who go into remission, the cancer often recurs, helping explain the low survival rate. Without any treatment, the average survival length after diagnosis is 1.5 months. With treatment, though, people can live longer or even recover. Overall, treatment will lead to long-term survival in 15–20% of people with CNS lymphoma. Newer treatments have prolonged survival. For example, research has shown that the median overall survival with high dose methotrexate is 25–55 months. However, some people live much longer, and doctors cannot predict how long someone will live or whether the cancer will recur. The outlook for people with CNS lymphoma varies among individuals. Factors that reduce the chance of a positive outlook include:
Lymphoma develops when immune system cells divide out of control. Scientists do not fully understand why some types of lymphoma enter the CNS or why some people develop lymphoma. Some risk factors that may increase the chances of developing non-Hodgkin lymphoma, including CNS lymphoma, include:
People with CNS lymphoma may not have any symptoms, or the symptoms might appear slowly. People who have symptoms may notice neurological changes, such as:
Doctors use a combination of tests to diagnose CNS lymphoma. These may include:
Unlike other forms of lymphoma, which doctors stage according to how much and how far they have spread, there is no standard staging system for CNS lymphoma. Instead, doctors look at various factors, such as the location of cancer in the body and a person’s overall health, to help them determine the best treatment plan. Doctors also assess whether the lymphoma is primary or secondary. Primary CNS lymphoma refers to lymphoma that initially appeared in the brain or spinal cord. Secondary means that the lymphoma has spread to the CNS from other parts of the body. Certain disease markers, including elevated levels of protein or lactate dehydrogenase in the cerebrospinal fluid, can affect a person’s outlook after diagnosis. The right treatment depends on many factors, including the location of the cancer and the person’s overall health and treatment goals. The main treatment options include:
About half of people with CNS lymphoma will experience a return of the cancer, usually within 5–10 years. When this cancer returns, the average overall survival is 2 months. CNS lymphoma is an aggressive cancer that affects the brain or spinal cord. Treatment options are improving rapidly, and the survival time is steadily improving. Prompt, aggressive treatment can prolong a person’s life and may cure the cancer. Anyone who is experiencing unexplained neurological symptoms should see a doctor as soon as possible to rule out this condition. Last medically reviewed on April 27, 2021
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