Disseminated lymphoblastic lymphoma in children and adolescents: results of the COG A5971 trial: a report from the Children's Oncology Group.

The Children's Oncology Group's A5971 trial examined central nervous system ( CNS) prophylaxis and early intensification in paediatric patients diagnosed with CNS-negative Stage III and IV lymphoblastic lymphoma. Using a 2 × 2 factorial design, the study randomized patients to Children's Cancer Group ( CCG) modified Berlin-Frankfurt-Muenster ( BFM) acute lymphoblastic leukaemia ( ALL) regimen with intensified intrathecal ( IT) methotrexate ( MTX) (Arm A1) or an adapted non-Hodgkin lymphoma/ BFM-95 therapy with high dose MTX in interim maintenance but no IT- MTX in maintenance (Arm B1) . Each cohort was randomized ± intensification (cyclophosphamide/anthracycline) (Arms A2/B2). For the 254 randomized patients, there was no difference in 5-year event-free survival ( EFS) for the four arms: Arm A1 , 80% [95% confidence interval ( CI) 67-89%] and Arm A2 , 81% (95% CI 69-89%); Arm B1 , 80% (95% CI 68-88%) and Arm B2 , 84% (95% CI 72-91%). The cumulative incidence of CNS relapse was 1·2%. Age <10 years and institutional imaging response at 2 weeks was associated with improved outcomes ( P < 0·001 and P = 0·014 for overall survival). CNS positive patients ( n = 12) did poorly [5-year EFS of 63% (95% CI 29-85%)]. For CNS-negative patients, there was no difference in outcome based on CNS prophylaxis ( IT- MTX versus HD- MTX) or with intensification. [ABSTRACT FROM AUTHOR]