[Diagnosis and treatment of anaplastic large-cell lymphoma in children and adolescents: a retrospective multicenter survey study].

Objective: To provide a descriptive review and improve our understanding of the clinical characteristics and treatment outcome of pediatric anaplastic large cell lymphoma (ALCL) in China. Method: The clinical data and outcomes of patients under 16 years of age with newly histopathologically-confirmed ALCL in 10 large single institutions in China between January 2009 and June 2014, were retrospectively analyzed.The event-free survival (EFS) was analyzed by the Kaplan-Meier method.The risk factors of disease progression or relapse were evaluated by logistic regression analysis.Significance was defined as P <0.05. Result: Of the 80 eligible patients (52 male, 28 female), the median age was 8.4 years (range, 1.3-15.7 years). Two patients (3%) were Stage Ⅰ, 9(11%) Stage Ⅱ, 64 (80 %) Stage Ⅲ, and 5(6%) Stage Ⅳ.The median time of follow-up was 25.2 months (range, 7.1-74.8 months), 55 patients survived without disease at the last of follow-up, the 3-year EFS was (65±6)%. Sixty-five patients (81%) were treated with the Chinese Children Cancer Group-B cell-non-Hodgkin Lymphoma 2010 protocol, regimen and 15 cases (19%) were treated with other regimens.The 3-year EFS were (68±5)% vs .(65±20)% ( P =0.21). The 3-year EFS was (57±7)% and (78±11)% for patients with or without B symptoms ( P =0.01). Twenty-four patients experienced disease progression or relapse. The median time from initial diagnosis to tumor failure was 7.0 months (ranged, 1.5-42.6 months) (median). At the last evaluation, there were 5 patients still alive after disease progression and relapse. By univariate analysis, sex ( P =0.04) and B symptoms ( P =0.00) were identified as risk factors of disease progression or relapse.Nevertheless, only B symptoms ( HR 5.60, 95% CI 1.47-21.27, P ≤0.05) were risk factors in multivariate analysis. Conclusion: The presenting features of children and adolescents with ALCL and efficiency in this study were similar to those reported by western countries.Refinement of therapeutic strategies to improve survival for patients with disease progression or relapse should be the priority in future clinical study.