Primary CNS Lymphoma is a rare form of Non-Hodgkin lymphoma that affects only the brain. 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.
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. In many parts of the world, the number of patients with Non-Hodgkin lymphoma have increased manifold, for reasons that are not entirely clear.
Nearly all cases of primary CNS lymphoma have an aggressive form of NHL. The common warning signs include headache, changes in personality and alertness. Symptoms increase fast, and most require seeing a doctor within a few weeks of the start of symptoms.
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. Complete removal of the tumor is not required. A CSF examination is done by taking a small amount of fluid out of your spinal cord through the back. Tests are then done to rule out disease in any other part of the body. Only then can it be called 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 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. See the full article on Primary CNS Lymphoma Treatment.
Before the development of effective chemotherapy that can reach the brain, the results of treatment of primary CNS lymphoma were poor. Outcomes have improved over the last few decades. Some patients can now be cured, and many others can now live longer than before.
