Primary CNS lymphoma: a clinician's guide.

Primary CNS lymphoma is a high malignant disease of the brain which can lead rapidly to death if diagnosis and/or the start of treatment is delayed. The age at time of diagnosis is a strong factor influencing prognosis so that in younger patients <65 years of age long-term survival may be achieved in a substantial percentage of patients, while in elderly patients long-term survival is seen much more rarely. First-line therapy consists of high-dose methotrexate-based (poly)chemotherapy. This review provides an overview of clinical presentation, steps to diagnosis, detailed information about current treatment concepts and specific information for particular clinical situations.