[Primary central nervous system lymphoma]

Primary central nervous system lymphoma is a rare cancer, with immunosuppression as sole known risk factor. However, it affects today mostly old immunocompetent patients. It usually presents with brain involvement, but ocular or meningeal involvement must be ruled out. Corticosteroids should be withheld if possible until the completion of brain biopsy, to obtain a histological diagnosis. PCNSL is highly chemosensitive and potentially curable. Remission can be achieved in the majority of cases using a high dose intravenous methotrexate-based polychemotherapy. Recent data suggests a benefit of adding rituximab. Relapses are frequent, but because of toxicity, consolidation treatment with either radiotherapy or high dose chemotherapy with autologous stem cell transplant is an option only in younger patients. Patients with primary central nervous system lymphoma must be treated by a specialized multidisciplinary team. In France, a network of expert centers covering the territory was created with the National Cancer Institute.