Thomas E Merchant1, Anne E Bendel2, Noah D Sabin1, Peter C Burger3, Dennis W Shaw4, Eric Chang5,6, Shengjie Wu1, Tianni Zhou7, David D Eisenstat8,9, Nicholas K Foreman10, Christine E Fuller11, Edwina Templeton Anderson1, Juliette Hukin12, Ching C Lau13,14, Ian F Pollack15, Fred H Laningham16, Robert H Lustig17, Floyd D Armstrong18, Michael H Handler10, Chris Williams-Hughes19, Sandra Kessel20, Mehmet Kocak1, David W Ellison1, Vijay Ramaswamy21. 1. 1 St Jude Children's Research Hospital, Memphis, TN. 2. 2 Children's Hospitals and Clinics of Minnesota, Minneapolis, MN. 3. 3 Johns Hopkins University/Sidney Kimmel Cancer Center, Baltimore, MD. 4. 4 Seattle Children's Hospital, Seattle, WA. 5. 5 University of Southern California, Los Angeles, CA. 6. 6 Norris Cancer Center, Los Angeles, CA. 7. 7 California State University, Long Beach, CA. 8. 8 University of Alberta and University of Alberta Hospital, Edmonton, Alberta, Canada. 9. 9 Edmonton Clinic Health, Edmonton, Alberta, Canada. 10. 10 Children's Hospital Colorado, Aurora, CO. 11. 11 Cincinnati Children's Hospital Medical Center, Cincinnati, OH. 12. 12 British Columbia Children's Hospital, Vancouver, British Columbia, Canada. 13. 13 Connecticut Children's Medical Center, Hartford, CT. 14. 15 University of Connecticut School of Medicine, Farmington, CT. 15. 16 Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA. 16. 17 Valley Children's Hospital, Madera, CA. 17. 18 University of California, San Francisco, School of Medicine, San Francisco, CA. 18. 19 University of Miami Miller School of Medicine-Sylvester Cancer Center, Miami, FL. 19. 20 Children's Oncology Group, Monrovia, CA. 20. 21 IROC Rhode Island, Lincoln, RI. 21. 22 Hospital for Sick Children, Toronto, Ontario, Canada.
Abstract
PURPOSE: The Children's Oncology Group trial ACNS0121 estimated event-free survival (EFS) and overall survival for children with intracranial ependymoma treated with surgery, radiation therapy, and-selectively-with chemotherapy. Treatment was administered according to tumor location, histologic grade, and extent of resection. The impacts of histologic grade, focal copy number gain on chromosome 1q, and DNA methylation profiles were studied for those undergoing surgery and immediate postoperative conformal radiation therapy (CRT). METHODS: ACNS0121 included 356 newly diagnosed patients (ages 1 to 21 years). Patients with classic supratentorial ependymoma were observed after gross total resection (GTR). Those undergoing subtotal resection received chemotherapy, second surgery, and CRT. The remaining patients received immediate postoperative CRT after near-total resection or GTR. CRT was administered with a 1.0-cm clinical target volume margin. The cumulative total dose was 59.4 Gy, except for patients who underwent GTR and were younger than age 18 months (who received 54 Gy). Patients were enrolled between October 2003 and September 2007 and were observed for 5 years. Supratentorial tumors were evaluated for RELA fusion; infratentorial tumors, for chromosome 1q gain. Classification of posterior fossa groups A and B was made by methylation profiles. RESULTS: The 5-year EFS rates were 61.4% (95% CI, 34.5% to 89.6%), 37.2% (95% CI, 24.8% to 49.6%), and 68.5% (95% CI, 62.8% to 74.2%) for observation, subtotal resection, and near-total resection/GTR groups given immediate postoperative CRT, respectively. The 5-year EFS rates differed significantly by tumor grade (P = .0044) but not by age, location, RELA fusion status, or posterior fossa A/posterior fossa B grouping. EFS was higher for patients with infratentorial tumors without 1q gain than with 1q gain (82.8% [95% CI, 74.4% to 91.2%] v 47.4% [95% CI, 26.0% to 68.8%]; P = .0013). CONCLUSION: The EFS for patients with ependymoma younger than 3 years of age who received immediate postoperative CRT and for older patients is similar. Irradiation should remain the mainstay of care for most subtypes.
PURPOSE: The Children's Oncology Group trial ACNS0121 estimated event-free survival (EFS) and overall survival for children with intracranial ependymoma treated with surgery, radiation therapy, and-selectively-with chemotherapy. Treatment was administered according to tumor location, histologic grade, and extent of resection. The impacts of histologic grade, focal copy number gain on chromosome 1q, and DNA methylation profiles were studied for those undergoing surgery and immediate postoperative conformal radiation therapy (CRT). METHODS:ACNS0121 included 356 newly diagnosed patients (ages 1 to 21 years). Patients with classic supratentorial ependymoma were observed after gross total resection (GTR). Those undergoing subtotal resection received chemotherapy, second surgery, and CRT. The remaining patients received immediate postoperative CRT after near-total resection or GTR. CRT was administered with a 1.0-cm clinical target volume margin. The cumulative total dose was 59.4 Gy, except for patients who underwent GTR and were younger than age 18 months (who received 54 Gy). Patients were enrolled between October 2003 and September 2007 and were observed for 5 years. Supratentorial tumors were evaluated for RELA fusion; infratentorial tumors, for chromosome 1q gain. Classification of posterior fossa groups A and B was made by methylation profiles. RESULTS: The 5-year EFS rates were 61.4% (95% CI, 34.5% to 89.6%), 37.2% (95% CI, 24.8% to 49.6%), and 68.5% (95% CI, 62.8% to 74.2%) for observation, subtotal resection, and near-total resection/GTR groups given immediate postoperative CRT, respectively. The 5-year EFS rates differed significantly by tumor grade (P = .0044) but not by age, location, RELA fusion status, or posterior fossa A/posterior fossa B grouping. EFS was higher for patients with infratentorial tumors without 1q gain than with 1q gain (82.8% [95% CI, 74.4% to 91.2%] v 47.4% [95% CI, 26.0% to 68.8%]; P = .0013). CONCLUSION: The EFS for patients with ependymoma younger than 3 years of age who received immediate postoperative CRT and for older patients is similar. Irradiation should remain the mainstay of care for most subtypes.
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