Treatment 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.
Bibliographical noteFunding Information:
LGR received research support from St. Baldrick’s Foundation and Hyundai Hope on Wheels; JPL received research support from the Lymphoma Foundation.
NLB has served as a consultant for Seattle Genetics, Forty Seven and KITE, and has received research funding from Seattle Genetics, Janssen, Pharmacyclics, Genentech, Pfizer, Celgene, Millennium, KITE, Merck, Bristol-Myers Squibb, Forty Seven and Affimed Therapeutics. BC has received research funding from Seattle Genetics, Immunomedics, Cel-gene, Genentech/Roche, Merck, Acerta Pharma, Pharma-cyclics, Janssen and Bristol-Myers Squibb. CC has served as a consultant for Infinity Pharmaceuticals and received research funding from Celgene. SMS has served as a consultant for Genentech/Roche, TG Therapeutics, Gilead Sciences, Phar-macyclics, NanoString Technologies, Genmab, Pharmacyclics, Forty Seven and Juno Therapeutics. JS has served as a consultant for Seattle Genetics and received research funding from Celgene, Seattle Genetics, MedImmune and Pharma-cyclics. RG has served as a consultant for Amgen. JPL has served as a consultant for Gilead, June, KITE, Genmab, NanoString, Regeneron, Abbvie, Sutro, Sunesis, Bristol-Myers Squibb and Genentech. All other authors have no conflicts of interest to report.
- non-Hodgkin lymphoma
- paediatric oncology
- primary mediastinal B-cell lymphoma