title: Long-term adverse outcomes in survivors of childhood bone sarcoma: the British Childhood Cancer Survivor Study.
abstract: BACKGROUND
  With improved survival, more bone sarcoma survivors are approaching middle age making it crucial to investigate the late effects of their cancer and its treatment. We investigated the long-term risks of adverse outcomes among 5-year bone sarcoma survivors within the British Childhood Cancer Survivor Study.
  
  
  METHODS
  Cause-specific mortality and risk of subsequent primary neoplasms (SPNs) were investigated for 664 bone sarcoma survivors. Use of health services, health and marital status, alcohol and smoking habits, and educational qualifications were investigated for survivors who completed a questionnaire.
  
  
  RESULTS
  Survivors were seven times more likely to experience all-cause mortality than expected, and there were substantial differences in risk depending on tumour type. Beyond 25 years follow-up the risk of dying from all-causes was comparable to the general population. This is in contrast to dying before 25 years where the risk was 12.7-fold that expected. Survivors were also four times more likely to develop a SPN than expected, where the excess was restricted to 5-24 years post diagnosis. Increased health-care usage and poor health status were also found. Nonetheless, for some psychosocial outcomes survivors were better off than expected.
  
  
  CONCLUSIONS
  Up to 25 years after 5-year survival, bone sarcoma survivors are at substantial risk of death and SPNs, but this is greatly reduced thereafter. As 95% of all excess deaths before 25 years follow-up were due to recurrences and SPNs, increased monitoring of survivors could prevent mortality. Furthermore, bone and breast SPNs should be a particular concern. Since there are variations in the magnitude of excess risk depending on the specific adverse outcome under investigation and whether the survivors were initially diagnosed with osteosarcoma or Ewing sarcoma, risks need to be assessed in relation to these factors. These findings should provide useful evidence for risk stratification and updating clinical follow-up guidelines.
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: M M
authors.initials: MM
authors.lastname: Fidler
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: C
authors.initials: C
authors.lastname: Frobisher
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: J
authors.initials: J
authors.lastname: Guha
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: K
authors.initials: K
authors.lastname: Wong
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: J
authors.initials: J
authors.lastname: Kelly
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: D L
authors.initials: DL
authors.lastname: Winter
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: E
authors.initials: E
authors.lastname: Sugden
authors.affiliation: Orthopaedic Department, Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ, UK.
authors.forename: R
authors.initials: R
authors.lastname: Duncan
authors.affiliation: NIHR University College London Hospitals Biomedical Research Centre, London NW1 2PG, UK.
authors.forename: J
authors.initials: J
authors.lastname: Whelan
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: R C
authors.initials: RC
authors.lastname: Reulen
authors.affiliation: Centre for Childhood Cancer Survivor Studies, School of Health and Population Sciences, Public Health Building, University of Birmingham, Birmingham B15 2TT, UK.
authors.forename: M M
authors.initials: MM
authors.lastname: Hawkins
mesh_terms: D000293:Adolescent; D001859:Bone Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012307:Risk Factors; D012509:Sarcoma / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D011795:Surveys and Questionnaires; D017741:Survivors; D006113:United Kingdom / Q000453:epidemiology