Primary central nervous system lymphoma

Opinion statement There is no class I evidence for any therapeutic option in primary central nervous system lymphoma (PCNSL). When possible, patients should be included in clinical trials. The role of surgery is restricted to stereotactic biopsy in order to gain material for histopathologic diagnosis. Radiotherapy alone is associated with a median survival of no more than 1.5 years; cure is exceptional. However, in patients aged younger than 60 years, cure is the therapeutic aim. Polychemotherapy based on high-dose methotrexate with deferred radiation results in long-term survival in most of these patients and possibly cure in a substantial fraction of these patients. With regard to chemotherapy in PCNSL, the following must be considered: 1) the most efficient drug in PCNSL is methotrexate at a dosage of at least 1.5 g/m² per single dose; 2) methotrexate alone will lead to complete remission in only some patients, whereas the combination of methotrexate with other drugs is more efficient; and 3) the value of additional intraventricular chemotherapy and the necessity of “consolidation” radiotherapy after response to chemotherapy are not yet defined. For patients aged older than 60 years, no curative regimen with acceptable toxicity has yet been established. The combination of radiotherapy with methotrexate-based chemotherapy leads to severe long-term neurotoxic sequelae, ie, cognitive dysfunction, in most older patients and in some patients aged younger than 60 years.