Primary lymphoma of the central nervous system is a rare cancerous tumor that develops from cells of the central nervous system, causing an increase in pressure within the skull and symptoms such as vision problems, headaches, and vomiting. Treatment options include chemotherapy, radiation therapy, and steroids, but long-term survival is rare.
A primary lymphoma of the central nervous system is a rare cancerous tumor that develops from cells of the central nervous system, which consists of the brain, eyes, and spinal cord. The cancer can also be described as primary CNS lymphoma, or PCNSL, for short. A primary cancer is one that originated where it is growing, while a secondary cancer is one that has spread somewhere else, so a primary CNS lymphoma is thought to develop from nerve cells in the central nervous system. It is not fully understood how this occurs, as lymphomas normally develop from lymphatic tissue, which is part of the immune system and is not found in the central nervous system. Although primary lymphoma of the central nervous system is rare, it is becoming more common in people with immunosuppression, where the immune system is weak, due to diseases such as AIDS or following an organ transplant.
The symptoms of a primary lymphoma of the central nervous system are mainly due to an increase in pressure within the skull. This can be caused by a blockage in the fluid flow surrounding the brain or directly by the growing tumor mass. An increase in pressure can lead to vision problems, headaches and vomiting. Other symptoms caused by the tumor may include increasing confusion and seizures. If certain areas of the brain are affected by lymphoma, problems with balance and coordination can develop, and occasionally one side of the body can become weak.
Most often, a primary lymphoma of the central nervous system is what is known as high-grade non-Hodgkin lymphoma, which means that it tends to grow rapidly and is more likely to spread than a low-grade cancer. Treatment for primary intracranial cancer varies depending on the location and size of the lymphoma and whether it has already spread. An otherwise healthy person may require different treatment from an immunosuppressed person. There is no single best treatment option, but chemotherapy can be used, sometimes in combination with radiation therapy, and steroids can lower the pressure within the skull by reducing swelling and tumor size.
The outlook for someone with primary central nervous system lymphoma varies depending on the individual and the type of treatment received, but long-term survival is rare. Generally, there is a 50 percent chance of surviving more than two years. Younger patients who enjoy better overall health have a more positive prognosis.
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