Primary CNS lymphoma (PCNSL) accounts for 3% of all primary brain tumors with a median age at onset of about 62 years. In the vast majority of cases, PCNSL presents as unifocal or multifocal enhancing lesions on MRI, frequently adjacent to the ventricles. Stereotactic biopsy is the diagnostic procedure of choice revealing high-grade malignant non-Hodgkin's B-cell lymphoma in more than 90% of cases. Therapy is not evidence based. When eligible, patients should be included in clinical trials. In patients younger than 60 years cure is the aim. Polychemotherapy based on high-dose methotrexate (MTX) or alternatively high-dose chemotherapy with autologous stem cell rescue should be offered to patients eligible for this regimens. For patients over 60 years of age no curative regimen with acceptable toxicity has yet been established. An MTX-based chemotherapy, for example, in combination with temozolomide, is recommended. The role of radiotherapy as part of the initial treatment is not established; however, the combination of radiotherapy with MTX-based chemotherapy potentially leads to severe long-term neurotoxic sequelae. Therefore, radiotherapy as part of the initial therapy is not recommended by the author outside clinical trials. At relapse or in cases of refractory disease, patients will frequently benefit of salvage therapy, which depends on the initial treatment.
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