Large-cell lymphoma arising in the mediastinum in children and adolescentsis associated with an excellent outcome: A Children's Cancer Group report

Purpose: Large-cell lymphoma (LCL) arising in the mediastinum (LCL-M) is a heterogeneous group of non-Hodgkin's lymphoma (NHL) that includes a-cell ly mphomas as well as T-cell lymphomas, including anaplastic LCL. LCL-M is wel l recognized in young adults but is less well characterized and infrequent in children and adolescents. Methods: A retrospective review of Children's Cancer Group therapeutic stud ies for nonlymphoblastic lymphomas (CCG-551, CCG-503, CCG-552, and CCG-5911 ) identified 20 patients with LCL-M, representing 7.2% of all LCLs classifi ed by central pathology review. Results: The patients ranged in age from 4 to 19 years (median, 12.5 years; mean, 12 years); 55% of the patients were male. Although a variety of chem otherapy regimens were used, response was excellent, with all 20 patients ( 100%) achieving a complete response. Four patients (20%) experienced relaps e locally or in distant sites including brain and kidney. One patient died of sepsis during therapy. For the 20 patients with LCL-M, the product-limit estimated 5-year event-free survival (EFS) and 5-year overall survival (OS ) rates are 75% +/- 10% and 85% +/- 8%, respectively For disseminated LCLs (192 cases), the EFS and OS rates were 50% +/- 4% and 63% +/- 4%, respectiv ely, which differ significantly from the those of the LCL-M cases (EFS, P = .025; OS, P =.034). The 5-year EFS and OS rates far patients with localized LCL (67 cases) were 92 +/- 3% and 97 +/- 2%, respectively. Conclusion: LCL-M is ct heterogeneous group of NHLs that makes up approxima tely 7.2% of LCL in children and adolescents. Response to therapy and 05 in this young age group seems excellent and superior to that of disseminated LCLs but inferior to that of other localized LCL. Future studies of LCL-M w ill evaluate short intense chemotherapy administered without radiation ther apy. (C) 2000 by American Society of Clinical Oncology.