Intensive chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: Final results of children's cancer group study 5941

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Abstract
Background Anaplastic large cell lymphoma (ALCL) is characterized by advanced disease at presentation (70–80% of pediatric cases) and accounts for 10–15% of all childhood lymphomas. Treatment strategies for pediatric ALCL vary from short pulse B‐NHL chemotherapy to prolonged leukemia like therapy. The optimal treatment strategy is unknown. Methods CCG‐5941 used a compressed aggressive multiagent T‐cell lineage chemotherapy regimen consisting of a 3‐week induction therapy (vincristine, prednisone, cyclophosphamide, daunomycin, asparaginase) followed by a 3‐week consolidation period (vincristine, prednisone, etoposide, 6‐thioguanine, cytarabine, asparaginase, methotrexate) followed by six courses of maintenance chemotherapy at 7‐week intervals (cyclophosphamide, 6‐thioguanine, vincristine, prednisone, doxorubicin, asparaginase, methotrexate etoposide, cytarabine). Total therapy was 48 weeks. Results Eighty‐six children (male 56%, female 44%) with non‐localized ALCL (CD30+) were treated. The majority of tumors were positive for ALK (90%) and of T lineage (83%). Extranodal disease was common (mediastinum 35%, skin 15%, lung 14%, bone 12%, bone marrow 13%, liver 6%, and other viscera 17%). Grade 4 neutropenia occurred in 82% of patients. The 5‐year EFS was 68% (95% CI of 57–78%) and the 5‐year OS was 80% (95% CI of 69–87%). There were 21 relapses and 4 toxic deaths as first events. Relapse occurred early with 17 (81%) relapses occurring within 2 years of diagnosis and 12 (57%) while receiving therapy. Univariate analysis for risk factors only identified bone marrow involvement predicting lower EFS (P = 0.03). Conclusions CCG‐5941 demonstrated efficacy similar to previously reported regimens but with significant hematologic toxicity. Pediatr Blood Cancer 2009;52:335–339.

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