Disseminated nonlymphoblastic lymphoma of childhood: A childrens cancer group study, CCG‐552

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Abstract
(1) Purpose: To assess the efficacy of a chemotherapy‐only regimen in pediatric patients with disseminated nonlymphoblastic lymphoma and acute B‐cell leukemia (B‐ALL).(2) Patients and Methods: Sixty‐eight eligible patients with previously untreated disseminated non‐lymphoblastic lymphoma were enrolled on a Childrens Cancer Group study. Therapy included cycles of chemotherapy, systemic and intrathecal (IT), ever 3 weeks for a total maximal duration of 57 weeks. Fifty‐five patients had small non‐cleaved cell lymphoma (SNCCL) and 13 had diffuse large cell lymphoma (DLCL). Forty‐seven were stage III, six were stage IV, and 15 had B‐ALL; 13 had central nervous system (CNS) involvement.(3) Results: Four year event‐free survival (EFS) was 53% (SE ± 12%). Stage III SNCCL patients with LDH < 500 IU/L achieved an improved EFS compared to other SNCCL patients (86% vs. 42% 4 year EFS, P = .072). The primary site of failure for advanced stage SNCCL patients was the CNS. All Ki‐1‐positive DLCL patients relapsed. Patterns of failure, time to relapse, and outcome following relapse differed between SNCCL and DLCL patients.(4) Conclusions: Advanced stage SNCCL requires better CNS‐directed chemotherapy to reduce the CNS failure rate; however, the achievement of durable disease‐free survival in four of 11 patients with CNS disease without use of cranial irradiation suggests merit for further evaluation of chemotherapy‐only strategies. DLCL patients do not need intensive CNS‐directed chemotherapy.

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