Narrative review: secondary central nervous system lymphoma

Abstract

: Secondary central nervous system (CNS) lymphomas (SCNSL) include the systemic lymphoproliferative diseases with CNS involvement at presentation or at relapse or at both stages of disease. Potentially all lymphoproliferative diseases can present or relapse in the CNS, although with a different incidence. While for some of these the management of CNS localization can be considered standard for others a worldwide consensus on the management and treatment lacks. The incidence of CNS relapse in diffuse large B-cell lymphoma (DLBCL) is about 5%, and it is possibly slightly reduced over the last decades. Two possible reasons of this reduction are: (I) the improvement of systemic disease control obtained with the addition of rituximab to the chemotherapy; (II) the advances in identifying patients at high risk of CNS relapse and the application of prophylaxis. However, many unanswered questions remain and there is not a worldwide consensus on the criteria identifying patients at high risk of CNS and on the standard prophylaxis therapy. Patients who develop SCNSL have a poor prognosis, and the optimal treatment is unknown, and indeed often unsatisfactory. In this manuscript we report the important advances of the knowledge of this rare and fatal disease, obtained in the last years, thanks to the development of multicenter collaborations. However, this disease remains still highly fatal and the discovery of more and more efficient therapy strategies is becoming a priority. New therapeutic strategies alternative to or in combination with chemotherapy such as target and immunomodulatory therapy are being addressed in future trials. In this regard, a more accurate knowledge of the molecular and biological characteristics of this malignancy is becoming a priority for the development of innovative therapies that will be firstly investigated in refractory/relapsing patients and, if efficient, successively incorporated as part of first-line treatment.