title: Risk adapted chemotherapy for localised Ewing's sarcoma of bone: the French EW93 study.
abstract: OBJECTIVE
  To determine whether a risk factor adapted chemotherapy would improve the outcome of non-metastatic bone Ewing's sarcoma.
  
  
  METHODS
  Standard risk tumours (SR, good histological response to chemotherapy or small unresected tumours) received the previous EW88 chemotherapy. Ifosfamide/etoposide (IE) were introduced after 3 courses of cyclophosphamide/doxorubicine when tumour regression was <50% or during consolidation therapy for the intermediate risk tumours (IR, intermediate histological response 5-30% residual cells or large unresected tumours >100ml). High risk tumours (HR, histological poor response >30% residual cells or clinical poor response <50% for unresectable tumours), received IE prior high dose busulfan/melphalan with stem cell rescue.
  
  
  RESULTS
  From 1993 to 1999, 214 patients were enrolled. 5 y-EFS and OS were 60% (95% confidence interval (CI), 53-66) and 69% (95% CI, 63-75), respectively. 116 (54%), 46 (21%), 48 (22%) patients were considered as SR, IR and HR of relapse, respectively. No advantage to IE was observed in the IR group. As compared to previous study, tumour with poor histological response to induction chemotherapy seemed to benefit from the consolidation strategy including busulfan/melphalan: EFS were 45% (95% CI, 30-60) and 20% (95% CI, 7-43) for EW93 and EW88, respectively. Despite a risk-adapted strategy, histological response to chemotherapy remains the main prognostic factor in resected tumours, while initial tumour volume is the main prognostic factor for unresected tumours.
  
  
  CONCLUSIONS
  These results showing a potential benefit of a consolidation strategy including busulfan/melphalan as compared to conventional chemotherapy needed confirmation by a randomised trial and were one of the bases of the ongoing EuroEwing99.
authors.affiliation: Department of Paediatric and Adolescent Oncology, Institut Gustave Roussy, Villejuif, France. Nathalie.GASPAR@igr.fr
authors.forename: Nathalie
authors.initials: N
authors.lastname: Gaspar
authors.forename: Annie
authors.initials: A
authors.lastname: Rey
authors.forename: Perrine Marec
authors.initials: PM
authors.lastname: Bérard
authors.forename: Jean
authors.initials: J
authors.lastname: Michon
authors.forename: Jean Claude
authors.initials: JC
authors.lastname: Gentet
authors.forename: Marie Dominique
authors.initials: MD
authors.lastname: Tabone
authors.forename: Henri
authors.initials: H
authors.lastname: Roché
authors.forename: Anne Sophie
authors.initials: AS
authors.lastname: Defachelles
authors.forename: Odile
authors.initials: O
authors.lastname: Lejars
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Plouvier
authors.forename: Claudine
authors.initials: C
authors.lastname: Schmitt
authors.forename: Binh
authors.initials: B
authors.lastname: Bui
authors.forename: Patrick
authors.initials: P
authors.lastname: Boutard
authors.forename: Sophie
authors.initials: S
authors.lastname: Taque
authors.forename: Martine
authors.initials: M
authors.lastname: Munzer
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Vannier
authors.forename: Dominique
authors.initials: D
authors.lastname: Plantaz
authors.forename: Natacha
authors.initials: N
authors.lastname: Entz-Werle
authors.forename: Natacha
authors.initials: N
authors.lastname: Enz-Werlé
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy*; D002648:Child; D002675:Child, Preschool; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D012307:Risk Factors; D012512:Sarcoma, Ewing / Q000188:drug therapy*; D016896:Treatment Outcome; D044465:White People; D055815:Young Adult