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Outcome of newly diagnosed children and adolescents with localized lymphoblastic lymphoma treated on Children's Oncology Group trial A5971: A report from the Children's Oncology Group
Author(s) -
Termuhlen Amanda M.,
Smith Lynette M.,
Perkins Sherrie L.,
Lones Mark,
Finlay Jonathan L.,
Weinstein Howard,
Gross Thomas G.,
Abromowitch Minnie
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24149
Subject(s) - medicine , immunophenotyping , lymphoblastic lymphoma , methotrexate , pediatrics , cog , regimen , acute lymphocytic leukemia , lymphoma , oncology , lymphoblastic leukemia , leukemia , immunology , antigen , immune system , t cell , artificial intelligence , computer science
Background Localized lymphoblastic lymphoma (LL) is rare in pediatric patients. We report the 5‐year event‐free survival (EFS) and overall survival (OS) for children and adolescents with localized LL treated on a uniform regimen based on Children's Cancer Group (CCG) leukemia therapy (COG A5971). Procedure From June 2000 to October 2005, the study enrolled 60 patients >12 months old with Murphy stages I or II LL. Central review confirmed 56 eligible patients. Treatment consisted of 24 months of CCG BFM without day 28 intrathecal methotrexate in maintenance therapy or prophylactic cranial radiation. Results Most patients had pre‐B immunophenotype (75%). At a median follow‐up of 5.9 years (range 1.4–9.3 years), the 5‐year EFS was 90% [95% confidence interval (CI), 78–96%] and the 5‐year OS was 96% (95% CI, 84–99%). Stage (I vs. II), immunophenotype, elevated LDH > institutional normal, or primary site did not impact outcome. Five relapses occurred—none in the CNS and none in patients with pre‐T lymphoblastic disease. Patients tolerated treatment well with no toxic deaths. Conclusion Outcomes of pediatric patients with localized LL treated with 2 years of intensive acute lymphoblastic leukemia (ALL)‐type therapy was excellent and is similar to the outcome for standard risk ALL treated less intensively. CNS prophylaxis was adequate with limited intrathecal methotrexate and no radiation. Future studies should identify biologic prognostic factors or biomarkers for pediatric patients with LL, explore less intensive treatment for patients with localized disease, and explore novel immunophenotype directed therapies. Pediatr Blood Cancer 2012; 59: 1229–1233. © 2012 Wiley Periodicals, Inc.