title: Long-term Outcomes and Complications in Pediatric Ewing Sarcoma.
abstract: OBJECTIVE
  The objective of this study was to determine treatment outcomes and long-term complications in pediatric patients with Ewing Sarcoma treated at the British Columbia Cancer Agency (BCCA).
  
  
  METHODS
  A retrospective chart review of 101 pediatric patients (<19 y old) with Ewing Sarcoma diagnosed between 1960 and 2005 was performed. The Kaplan-Meier survival analysis and Cox regression multivariate analysis were used to assess prognostic factors for overall survival (OS) and event-free survival (EFS).
  
  
  RESULTS
  The median age at diagnosis was 11 years and the median follow-up for nondeceased patients was 13.5 years. The most common primary tumor locations were lower extremity (33%), pelvis (24%), and thorax (18%). Fifty percent of patients received surgery, 79% radiotherapy and 94% chemotherapy. The 5-year OS and EFS for patients with localized disease was 85% and 73% and for metastatic disease was 27% (P<0.0001) and 28% (P<0.0001), respectively. Metastatic disease was an independent predictor of lower OS (hazard ratio [HR], 9.5; 95% confidence interval [CI],4.7-19.4; P<0.0001) and EFS (HR, 4.9; 95% CI, 2.7-8.8; P<0.0001). Extremity tumor location was an independent predictor for improved OS (HR, 0.4; 95% CI, 0.2-0.9; P=0.03). The majority (77%) of long-term survivors (≥5 y) had long-term complications; the most common were musculoskeletal abnormalities (50%) and cardiac toxicity (28%). The actuarial second neoplasm risk was 5% at 10 years.
  
  
  CONCLUSIONS
  Ewing sarcoma patients with localized disease had excellent treatment outcomes at the BCCA. However, the majority of patients had chronic complications from treatment. This study validates the need for long-term follow-up of Ewing Sarcoma survivors for management of late effects.
authors.affiliation: *Department of Surgery, Faculty of Medicine, University of British Columbia †Department of Radiation Oncology, British Columbia Cancer Agency-Vancouver Centre ∥Division of Pediatric Hematology/Oncology/BMT, British Columbia Children's Hospital, Vancouver, BC ‡Department of Oncology, Northeast Cancer Centre, Sudbury, ON, Canada §Pediatric Oncology Group of Ontario, Toronto, ON, Canada.
authors.forename: Sarah N
authors.initials: SN
authors.lastname: Hamilton
authors.forename: Ryan
authors.initials: R
authors.lastname: Carlson
authors.forename: Haroon
authors.initials: H
authors.lastname: Hasan
authors.forename: Shahrad R
authors.initials: SR
authors.lastname: Rassekh
authors.forename: Karen
authors.initials: K
authors.lastname: Goddard
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D001955:British Columbia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D017741:Survivors; D016896:Treatment Outcome; D055815:Young Adult