Emerging novel agents for patients with advanced Ewing sarcoma: a report from the Children’s Oncology Group (COG) New Agents for Ewing Sarcoma Task Force [version 1; peer review: 3 approved]
Kelly Bailey,
Carrye Cost,
Ian Davis,
Julia Glade-Bender,
Patrick Grohar,
Peter Houghton,
Michael Isakoff,
Elizabeth Stewart,
Nadia Laack,
Jason Yustein,
Damon Reed,
Katherine Janeway,
Richard Gorlick,
Stephen Lessnick,
Steven DuBois,
Pooja Hingorani
Affiliations
Kelly Bailey
Division of Pediatric Hematology/Oncology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
Carrye Cost
Center for Cancer and Blood Disorders, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA
Ian Davis
Departments of Pediatrics and Genetics, Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC, USA
Julia Glade-Bender
Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
Patrick Grohar
Departement of Pediatrics, Van Andel Institute, Helen De Vos Children’s Hospital and Michigan State University, Grand Rapids, MI, USA
Peter Houghton
Greehey Children’s Cancer Research Institute, University of Texas Health Science Center, San Antonio, TX, USA
Michael Isakoff
Center for Cancer and Blood Disorders, Connecticut Children’s Medical Center, Hartford, CT, USA
Elizabeth Stewart
Department of Oncology, St. Jude Children’s Research Hospital, Memphis, TN, USA
Nadia Laack
Department of Radiation Oncology, Mayo Clinic, Rochester, MN, USA
Jason Yustein
The Faris D. Virani Ewing Sarcoma Center at the Texas Children’s Cancer Center, Baylor College of Medicine, Houston, TX, USA
Damon Reed
AYA Program, Moffitt Cancer Center, Tampa, FL, USA
Katherine Janeway
Dana-Farber/Boston Children’s Cancer and Blood Disorders Center and Harvard Medical School, Boston, MA, USA
Richard Gorlick
Division of Pediatrics, University of Texas MD Anderson Cancer Center, Houston, TX, USA
Stephen Lessnick
Center for Childhood Cancer and Blood Diseases, Research Institute at Nationwide Children’s Hospital, Columbus, OH, USA
Steven DuBois
Dana-Farber/Boston Children’s Cancer and Blood Disorders Center and Harvard Medical School, Boston, MA, USA
Pooja Hingorani
Center for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, AZ, USA
Ewing sarcoma is a small round blue cell malignancy arising from bone or soft tissue and most commonly affects adolescents and young adults. Metastatic and relapsed Ewing sarcoma have poor outcomes and recurrences remain common. Owing to the poor outcomes associated with advanced disease and the need for a clear research strategy, the Children’s Oncology Group Bone Tumor Committee formed the New Agents for Ewing Sarcoma Task Force to bring together experts in the field to evaluate and prioritize new agents for incorporation into clinical trials. This group’s mission was to evaluate scientific and clinical challenges in moving new agents forward and to recommend agents and trial designs to the Bone Tumor Committee. The task force generated a framework for vetting prospective agents that included critical evaluation of each drug by using both clinical and non-clinical parameters. Representative appraisal of agents of highest priority, including eribulin, dinutuximab, cyclin-dependent kinase 4 and 6 (CDK4/6) inhibitors, anti-angiogenic tyrosine kinase inhibitors, and poly-ADP-ribose polymerase (PARP) inhibitors, is described. The task force continues to analyze new compounds by using the paradigm established.