Primary central nervous system lymphoma in children.

Primary central nervous system lymphoma (PCNSL) is a very rare brain tumor in children, and the optimal management and prognosis of such patients have yet to be defined. In this study, the incidence rate, clinical features, diagnosis, and treatment of childhood PCNSL are reviewed. Except for human immunodeficiency virus-related PCNSL, the prognosis for patients with this tumor type is significantly better in children than in adults. In the absence of prospective studies, it is very difficult to determine the true incidence and the best therapeutic strategy for this rare entity. The majority of children with PCNSL, however, can achieve long-term remissions with intensive chemotherapy alone (an estimated 70% 5-year event-free survival rate), and cranial irradiation can be reserved for relapse of the disease. Further progress in the management of childhood PCNSL will require prospective multinational studies.