title: Quality of Survivorship in a Rare Disease: Clinicofunctional Outcome and Physical Activity in an Observational Cohort Study of 618 Long-Term Survivors of Ewing Sarcoma.
abstract: Purpose Significantly improved survival rates in patients with Ewing sarcoma have raised interest in accessing the quality of long-term survivorship. In this study, subjective and objective measurement tools, preclassified as physical or mental scores, were used to assess clinicofunctional outcome and physical activity after intensive bone tumor treatment. Methods Long-term outcome of 618 survivors from consecutive Ewing sarcoma trials was assessed by the Toronto Extremity Salvage Score, Short-Form Health Survey (SF-36), Brief Symptom Inventory (BSI), and Rosenberg Self-Esteem Scale questionnaires and by the accelerometric StepWatch 3 Activity Monitor. Prospective measurements were correlated retrospectively with standardized primary trial data. Results were compared with 316 nonrandom healthy peers by using effect sizes ( d). Median observation time was 12.9 years from primary diagnosis (range, 3.7 to 31.2 years). Results Absolute subjective scores were moderate to good for survivors. Compared with control subjects, unfavorable outcome was shown on physical Toronto Extremity Salvage Score, SF-36 Physical Component Summary, and BSI-Somatization scales (| d| ≥ 0.50; P < .01), in contrast to SF-36 Mental Component Summary, BSI-Anxiety, BSI-Depression, and Rosenberg Self-Esteem Scale mental scales (| d| ≤ 0.31). Survivors were less active than control subjects, as demonstrated by a step count difference of 1,742 steps per day ( d = -0.43; P < .01); however, on average, the recommended level for an active lifestyle was achieved (≥ 10,000 steps). Location of pelvic tumor was the major inferior disease-specific prognostic factor in physical scores ( P < .01), whereas nondisease-specific inferior factors in questionnaires were older age and female sex ( P < .01). Conclusion Survivors of Ewing sarcoma apparently returned to a normal life with minor limitations. Observed reductions in physical scores should be a focus in future research to optimize treatment strategies to reduce a negative impact on the quality of survivorship.
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Andreas
authors.initials: A
authors.lastname: Ranft
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Corinna
authors.initials: C
authors.lastname: Seidel
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Christiane
authors.initials: C
authors.lastname: Hoffmann
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Michael
authors.initials: M
authors.lastname: Paulussen
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Ann-Christin
authors.initials: AC
authors.lastname: Warby
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Henk
authors.initials: H
authors.lastname: van den Berg
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Ruth
authors.initials: R
authors.lastname: Ladenstein
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Claudia
authors.initials: C
authors.lastname: Rossig
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Uta
authors.initials: U
authors.lastname: Dirksen
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Dieter
authors.initials: D
authors.lastname: Rosenbaum
authors.affiliation: Andreas Ranft, Christiane Hoffmann, Ann-Christin Warby, Claudia Rossig, Uta Dirksen, and Herbert Juergens, University Children's Hospital; Corinna Seidel, and Dieter Rosenbaum, University Hospital Muenster; Michael Paulussen, Witten/Herdecke University, Datteln, Germany; Henk van den Berg, Emma Children's Hospital, Amsterdam, the Netherlands; and Ruth Ladenstein, Children's Cancer Research Institute, Wien, Austria.
authors.forename: Herbert
authors.initials: H
authors.lastname: Juergens
mesh_terms: D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000503:physiopathology* / Q000523:psychology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015444:Exercise; D005260:Female; D006801:Humans; D008297:Male; D011379:Prognosis; D011788:Quality of Life; D012512:Sarcoma, Ewing / Q000503:physiopathology* / Q000523:psychology*; D017741:Survivors; D055815:Young Adult