title: Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies.
abstract: BACKGROUND
  In the multicenter European Intergroup Cooperative Ewing's Sarcoma Studies, localized Ewing tumors of bone were treated by combination chemotherapy with surgery and/or radiotherapy. Patients with primary metastases (pm-pts) were treated in high risk protocols.
  
  
  METHODS
  One hundred seventy-seven pm-pts were registered from January 1990 to December 1995, 171 were evaluable for survival analyses. Thirty-six pm-pts received myeloablative megatherapy with stem cell rescue following conventional treatment. Bilateral whole lung irradiation (WLI) was administered in 57 pm-pts with pulmonary involvement. Event-free survival (EFS) rates were estimated by Kaplan-Meier analysis. Prognostic factors were identified by log-rank statistics, Cox procedures and logistic regression.
  
  
  RESULTS
  Eighty-nine deaths were recorded by 1 February 1997, EFS four years after diagnosis for all 171 pm-pts was 0.27. EFS for isolated lung metastases was 0.34, for bone/bone marrow (BM) metastases, 0.28, and for combined lung plus bone/BM metastases, 0.14 (P < 0.005). WLI improved outcome in case of isolated pulmonary involvement (0.40 vs. 0.19, P < 0.05). In pm-pts with combined pulmonary/skeletal metastases, intensification by megatherapy and/or WLI improved EFS from 0.00 to 0.27 (P = 0.0001).
  
  
  CONCLUSIONS
  EFS four years after diagnosis in patients with disseminated Ewing tumors is 0.27. Whole lung irradiation and megatherapy improve outcome in subgroups of patients with disseminated Ewing tumors is 0.27. Whole lung irradiation and megatherapy improve outcome in subgroups of patients with disseminated Ewing disease.
authors.affiliation: Department of Pediatric Oncology, University of Münster, Germany. paulusm@uni-muenster.de
authors.forename: M
authors.initials: M
authors.lastname: Paulussen
authors.forename: S
authors.initials: S
authors.lastname: Ahrens
authors.forename: S
authors.initials: S
authors.lastname: Burdach
authors.forename: A
authors.initials: A
authors.lastname: Craft
authors.forename: B
authors.initials: B
authors.lastname: Dockhorn-Dworniczak
authors.forename: J
authors.initials: J
authors.lastname: Dunst
authors.forename: B
authors.initials: B
authors.lastname: Fröhlich
authors.forename: W
authors.initials: W
authors.lastname: Winkelmann
authors.forename: A
authors.initials: A
authors.lastname: Zoubek
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000704:Analysis of Variance; D001859:Bone Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D016015:Logistic Models; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D008297:Male; D015337:Multicenter Studies as Topic; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D016609:Neoplasms, Second Primary / Q000401:mortality* / Q000628:therapy; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000401:mortality* / Q000473:pathology / Q000556:secondary* / Q000628:therapy; D017678:Sex Distribution; D016019:Survival Analysis