• Lisa Giulino-Roth, Tara O'Donohue, Zhengming Chen, Nancy L Bartlett, Ann LaCasce, William Martin-Doyle, Matthew J Barth, Kimberly Davies, Kristie A Blum, Beth Christian, Carla Casulo, Sonali M Smith, James Godfrey, Amanda Termuhlen, Matthew J Oberley, Sarah Alexander, Sheila Weitzman, Burton Appel, Benjamin Mizukawa, Jakub Svoboda, Zeinab Afify, Melinda Pauly, Hema Dave, Rebecca Gardner, Deborah M Stephens, William A Zeitler, Christopher Forlenza, Jennifer Levine, Michael E Williams, Jody L Sima, Catherine M Bollard, and John P Leonard.
• Department of Pediatrics, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
• Br. J. Haematol. 2017 Dec 1; 179 (5): 739-747.

AbstractTreatment with dose-adjusted EPOCH (etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone) chemotherapy and rituximab (DA-EPOCH-R) has become the standard of care for primary mediastinal B-cell lymphoma (PMBCL) at many institutions despite limited data in the multi-centre setting. We report a large, multi-centre retrospective analysis of children and adults with PMBCL treated with DA-EPOCH-R to characterize outcomes and evaluate prognostic factors. We assessed 156 patients with PMBCL treated with DA-EPOCH-R across 24 academic centres, including 38 children and 118 adults. All patients received at least one cycle of DA-EPOCH-R. Radiation therapy was administered in 14·9% of patients. With median follow-up of 22·6 months, the estimated 3-year event-free survival (EFS) was 85·9% [95% confidence interval (CI) 80·3-91·5] and overall survival was 95·4% (95% CI 91·8-99·0). Outcomes were not statistically different between paediatric and adult patients. Thrombotic complications were reported in 28·2% of patients and were more common in paediatric patients (45·9% vs. 22·9%, P = 0·011). Seventy-five per cent of patients had a negative fluorodeoxyglucose positron emission tomography (FDG-PET) scan at the completion of DA-EPOCH-R, defined as Deauville score 1-3. Negative FDG-PET at end-of-therapy was associated with improved EFS (95·4% vs. 54·9%, P < 0·001). Our data support the use of DA-EPOCH-R for the treatment of PMBCL in children and adults. Patients with a positive end-of-therapy FDG-PET scan have an inferior outcome.© 2017 John Wiley & Sons Ltd.

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