[Primary cerebral non-Hodgkin lymphoma in non-immunocompromised subjects]

Abstract

Cerebral non Hodgkin's lymphomas (NHL) of immunocompetent patients are rare disease (1% of all NHL), but their incidence has been steadily increasing in the last 20 years. Most cerebral NHL are diffuse large cell NHL with a B phenotype. These tumors are most often localized in the central nervous system, the eye or meninges; other systemic tumor sites are rare, either at initial diagnosis and relapse. The overall survival of primary cerebral lymphoma (PCL) is poor in the published retrospective series with a median survival in the range of 12 to 16 months and a 5 year survival between 5 et 20%. PCL are however curable brain tumors, and the outcome of these patients has probably improved in the past 10 years, using a multidisciplinary therapeutic approach. Complete resection of the tumor dose not improve the outcome of these patients. The treatment of PCL relies on combinations of chemotherapy and radiotherapy, but the type of regimen and the dose to the brain are still a matter of debate, in particular because of late neurological sequelae. Multicentric prospective clinical trials in France and Europe will provide answers to these questions in the next years.