Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R

Lisa Giulino-Roth^{1

2}, Tara O'Donohue¹, Zhengming Chen³, Nancy L Bartlett⁴, Ann LaCasce⁵, William Martin-Doyle⁶, Matthew J Barth⁷, Kimberly Davies⁸, Kristie A Blum⁹, Beth Christian⁹, Carla Casulo¹⁰, Sonali M Smith¹¹, James Godfrey¹¹, Amanda Termuhlen¹², Matthew J Oberley¹³, Sarah Alexander¹⁴, Sheila Weitzman¹⁴, Burton Appel¹⁵, Benjamin Mizukawa¹⁶, Jakub Svoboda¹⁷, Zeinab Afify¹⁸, Melinda Pauly^{19

20}, Hema Dave²¹, Rebecca Gardner²², Deborah M Stephens²³, William A Zeitler²⁴, Christopher Forlenza²⁵, Jennifer Levine²⁶, Michael E Williams²⁷, Jody L Sima²⁸, Catherine M Bollard²¹, John P Leonard²

Affiliations

¹ Department of Pediatrics, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
² Department of Medicine, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
³ Healthcare Policy and Research Meyer Cancer Center, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
⁴ Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
⁵ Medical Oncology, Dana Farber Cancer Institute, Boston, MA, USA.
⁶ Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.
⁷ Department of Pediatrics, Roswell Park Cancer Institute and University at Buffalo, Buffalo, NY, USA.
⁸ Department of Pediatric Oncology, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, MA, USA.
⁹ Division of Hematology, The Ohio State University and Arthur G. James Cancer Hospital, Columbus, OH, USA.
¹⁰ Department of Medicine, University of Rochester Medical Center, Rochester, NY, USA.
¹¹ Department of Medicine, University of Chicago, Chicago, IL, USA.
¹² Department of Pediatrics, Keck School of Medicine, Los Angeles, CA, USA.
¹³ Department of Pathology and Laboratory Medicine, Keck School of Medicine at the University of Southern California, Children's Hospital Los Angeles, Los Angeles, CA, USA.
¹⁴ Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
¹⁵ Institute for Pediatric Cancer & Blood Disorders, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ, USA.
¹⁶ Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
¹⁷ Lymphoma Program, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA, USA.
¹⁸ Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
¹⁹ Department of Pediatrics, Emory University, Atlanta, GA, USA.
²⁰ Aflac Cancer & Blood Disorders Center at Children's Healthcare of Atlanta, Atlanta, GA, USA.
²¹ Department of Pediatrics, The George Washington University and Children's National Health System, Washington, DC, USA.
²² Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
²³ Department of Medicine, University of Utah, Salt Lake City, UT, USA.
²⁴ Department of Medicine, University of Iowa, Iowa City, IA, USA.
²⁵ Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
²⁶ Department of Pediatrics, Columbia University Medical Center and New York Presbyterian Hospital, New York, NY, USA.
²⁷ Hematology/Oncology Division and Cancer Center, University of Virginia School of Medicine, Charlottesville, VA, USA.
²⁸ Department of Pediatrics, Upstate Medical University, Syracuse, NY, USA.

PMID: 29082519
PMCID: PMC6650639
DOI: 10.1111/bjh.14951

Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R

Lisa Giulino-Roth et al. Br J Haematol. 2017 Dec.

. 2017 Dec;179(5):739-747.

doi: 10.1111/bjh.14951. Epub 2017 Oct 29.

Authors

Affiliations

¹ Department of Pediatrics, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
² Department of Medicine, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
³ Healthcare Policy and Research Meyer Cancer Center, Weill Cornell Medicine and New York Presbyterian Hospital, New York, NY, USA.
⁴ Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.
⁵ Medical Oncology, Dana Farber Cancer Institute, Boston, MA, USA.
⁶ Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA.
⁷ Department of Pediatrics, Roswell Park Cancer Institute and University at Buffalo, Buffalo, NY, USA.
⁸ Department of Pediatric Oncology, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, MA, USA.
⁹ Division of Hematology, The Ohio State University and Arthur G. James Cancer Hospital, Columbus, OH, USA.
¹⁰ Department of Medicine, University of Rochester Medical Center, Rochester, NY, USA.
¹¹ Department of Medicine, University of Chicago, Chicago, IL, USA.
¹² Department of Pediatrics, Keck School of Medicine, Los Angeles, CA, USA.
¹³ Department of Pathology and Laboratory Medicine, Keck School of Medicine at the University of Southern California, Children's Hospital Los Angeles, Los Angeles, CA, USA.
¹⁴ Division of Haematology/Oncology, Hospital for Sick Children, Toronto, ON, Canada.
¹⁵ Institute for Pediatric Cancer & Blood Disorders, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ, USA.
¹⁶ Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
¹⁷ Lymphoma Program, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA, USA.
¹⁸ Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
¹⁹ Department of Pediatrics, Emory University, Atlanta, GA, USA.
²⁰ Aflac Cancer & Blood Disorders Center at Children's Healthcare of Atlanta, Atlanta, GA, USA.
²¹ Department of Pediatrics, The George Washington University and Children's National Health System, Washington, DC, USA.
²² Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
²³ Department of Medicine, University of Utah, Salt Lake City, UT, USA.
²⁴ Department of Medicine, University of Iowa, Iowa City, IA, USA.
²⁵ Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, NY, USA.
²⁶ Department of Pediatrics, Columbia University Medical Center and New York Presbyterian Hospital, New York, NY, USA.
²⁷ Hematology/Oncology Division and Cancer Center, University of Virginia School of Medicine, Charlottesville, VA, USA.
²⁸ Department of Pediatrics, Upstate Medical University, Syracuse, NY, USA.

PMID: 29082519
PMCID: PMC6650639
DOI: 10.1111/bjh.14951

Abstract

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.

Keywords: DA-EPOCH-R; non-Hodgkin lymphoma; paediatric oncology; primary mediastinal B-cell lymphoma.

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Figures

**Figure 1:**
Kaplan-Meier estimate of (A) event-free survival (EFS); (B) overall survival (OS) for paediatric and adult patients.

**Figure 2:**
Kaplan-Meier estimate of (A) event-free survival (EFS); (B) overall survival (OS) by end-of-therapy fluorodeoxyglucose positron emission tomography (PET) Deauville Score

See this image and copyright information in PMC

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Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R

Affiliations

Outcomes of adults and children with primary mediastinal B-cell lymphoma treated with dose-adjusted EPOCH-R

Authors

Affiliations

Abstract

Figures

References

Publication types

MeSH terms

Substances

Supplementary concepts

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources