Therapeutic management of primary CNS lymphoma in immunocompetent patients

The best therapeutic management in primary CNS lymphomas remains to be defined because of the small size and short follow-up of retrospective series, the methodological pitfalls and limited number of prospective studies, and the paucity of randomized trials. The purpose of this article is to analyze, discuss and summarize the current therapeutic approaches, namely chemotherapy or radiotherapy as exclusive treatment, combined treatment, most commonly used drugs, intrathecal chemotherapy and consolidation radiotherapy and to provide recommendations for ordinary clinical practice. Some important therapeutic issues such as the management of intraocular lymphomas, elderly patients and patients without histological diagnosis, as well as the relevance of salvage therapy as a playground for the evaluation of new drugs are also analyzed. Finally, the main open questions as well as current and expected investigation trends are discussed.