title: Italian Cooperative Study for the treatment of children and young adults with localized Ewing sarcoma of bone: a preliminary report of 6 years of experience.
abstract: BACKGROUND
  In 1991, the Italian Association for Pediatric Hematology-Oncology and the National Council of Research (CNR) initiated an Italian Cooperative Study (SE 91-CNR Protocol) with the main objective of improving the overall survival (SUR) and the event free survival (EFS) of children and young adults with localized Ewing sarcoma and primitive neuroectodermal tumors of bone compared with a previous study (IOR/Ew2 Protocol).
  
  
  METHODS
  Between November 1991 and November 1997, 165 patients were enrolled in this study, 160 of whom were evaluable. The patients were treated with a multimodal approach characterized by intensified chemotherapy, hyperfractionated and accelerated radiation therapy, and the addition of ifosfamide and etoposide to standard chemotherapy with vincristine, actinomycin-D, doxorubicin, and cyclophosphamide.
  
  
  RESULTS
  After a median follow-up of 37 months, 126 of the 160 evaluable patients remained free of disease recurrence. Thirty-one patients developed a disease recurrence (20 with disseminated disease).
  
  
  CONCLUSIONS
  The 3-year SUR and EFS rates found in the current study (83.6% and 77.8%, respectively) may be considered satisfactory. Only age at diagnosis < or =14 years and a good histologic response appeared to affect the outcome of patients with localized Ewing sarcoma positively. These results appear to demonstrate the efficacy of the addition of ifosfamide in induction chemotherapy to four-drug standard combination chemotherapy, as confirmed by the improved outcome in terms of 3-year EFS reported in the SE 91-CNR Protocol compared with the IOR/Ew2 Protocol (77.8% vs. 60.7%). In addition, the better outcome also could be explained by the change in treatment strategy with a trend toward the use of more surgery than radiation therapy compared with the authors' previous protocol.
authors.affiliation: Department of Pediatrics, University of Bologna, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Rosito
authors.forename: A F
authors.initials: AF
authors.lastname: Mancini
authors.forename: R
authors.initials: R
authors.lastname: Rondelli
authors.forename: M E
authors.initials: ME
authors.lastname: Abate
authors.forename: A
authors.initials: A
authors.lastname: Pession
authors.forename: L
authors.initials: L
authors.lastname: Bedei
authors.forename: G
authors.initials: G
authors.lastname: Bacci
authors.forename: P
authors.initials: P
authors.lastname: Picci
authors.forename: M
authors.initials: M
authors.lastname: Mercuri
authors.forename: P
authors.initials: P
authors.lastname: Ruggieri
authors.forename: G
authors.initials: G
authors.lastname: Frezza
authors.forename: M
authors.initials: M
authors.lastname: Campanacci
authors.forename: G
authors.initials: G
authors.lastname: Paolucci
mesh_terms: D000293:Adolescent; D000328:Adult; D000671:Amputation, Surgical; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002985:Clinical Protocols; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D011183:Postoperative Complications; D012074:Remission Induction; D012512:Sarcoma, Ewing / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D016019:Survival Analysis