A case series of pediatric primary central nervous system lymphoma: favorable outcome without cranial irradiation

Abstract

Purpose: To describe the outcome of childhood primary central nervous system lymphoma (PCNSL) treated with chemotherapy alone or with chemotherapy plus cranial radiotherapy (CRT).

Methods: Retrospective chart review of children with PCNSL at six tertiary care pediatric centers.

Results: Eight immunocompetent and four immunocompromised children were included. Ten children received chemotherapy alone without CRT, with most receiving high-dose methotrexate and high-dose cytarabine. Five year event-free survival (EFS) in this group was 70.0 +/- 14.5%. Two children received chemotherapy plus CRT; one relapsed and died while the other is alive in remission. Three children died, including two from relapsed disease. The other child with human immunodeficiency virus infection died of an opportunistic infection while in remission following chemotherapy alone.

Conclusions: Most children with PCNSL can achieve long-term remissions with chemotherapy alone without CRT. Multi-center prospective studies are needed to confirm or refute these results in a larger number of patients.