title: Validation of a multi-modal treatment protocol for Ewing sarcoma--a report from the polish pediatric oncology group.
abstract: BACKGROUND
  Ewing sarcoma (ES) is the second most common paediatric malignant bone tumor. Advances in multi-disciplinary care have resulted in significant improvement in cure rates over the last decades. However, the generalization of those results in countries traditionally excluded from large cooperative trials has yet to be demonstrated. We report the results of modern multi-disciplinary care for patients with ES in Poland.
  
  
  METHODS
  One hundred and thirty-two patients with ES were treated using modern multi-modal therapy during the period 2000-2009. Overall survival was estimated by Kaplan-Meier methods and compared using long-rank test and Cox models. Factors predictive of outcome in our setting were analyzed to identify distinct risk groups that could help identify areas for improvement.
  
  
  RESULTS
  The median age at the time of diagnosis was 12.3 years. With a median follow-up of 5.0 years, the 5-year event-free survival (EFS) and OS estimates for localized disease were 54.88% and 68.29%, respectively. For patients with metastatic disease, 5-year EFS and OS estimates were 36% and 42%, respectively. There was no correlation between age and stage or site. Patients with localized, non-pelvic disease had better outcome than patients with axial tumors (71% vs. 44%, respectively, P = 0.00073). Treatment failure was associated with stage, pelvic primary, poor histological response, and type of local control.
  
  
  CONCLUSIONS
  Successful treatment of ES requires optimal systemic and local therapy. We were able to replicate the results of modern multi-modal protocols. Validation of current treatment protocols in countries with more limited cancer treatment resources is required.
authors.affiliation: Department of Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Raciborska
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Bilska
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Drabko
authors.forename: Radosław
authors.initials: R
authors.lastname: Chaber
authors.forename: Grażyna
authors.initials: G
authors.lastname: Sobol
authors.forename: Monika
authors.initials: M
authors.lastname: Pogorzała
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Wyrobek
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Połczyńska
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Rogowska
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.forename: Wojciech
authors.initials: W
authors.lastname: Wożniak
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D008297:Male; D009367:Neoplasm Staging; D010372:Pediatrics; D011044:Poland; D011183:Postoperative Complications; D011379:Prognosis; D011879:Radiotherapy Dosage; D012512:Sarcoma, Ewing / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003520:Cyclophosphamide; D007069:Ifosfamide
keywords: Ewing sarcoma; children; survival; treatment