title: Developing a prognostic model for localized Ewing sarcoma family of tumors: A single institutional experience of 224 cases treated with uniform chemotherapy protocol.
abstract: BACKGROUND
  Data on patients with localized Ewing sarcoma family of tumors (ESFT) who have received a uniform chemotherapy protocol are minimal.
  
  
  METHODS
  This is a single institutional review of patients with ESFT treated between June 2003 and November 2011.
  
  
  RESULTS
  224/374 (60%) patients with ESFT presented with localized disease; median age was 15 years (range: 0.1-55). Ninety-nine patients underwent surgery of which 50 received adjuvant radiotherapy; 80 patients received radical radiotherapy following neoadjuvant chemotherapy. At median follow-up of 40.2 months (range: 1.3-129), 5-year EFS, OS, and local-control-rate, were 36.8 ± 3.6%, 52.4 ± 4.3%, and 63 ± 4.3%, respectively. In multivariate analysis, tumor diameter > 8 cm (P = 0.03), symptom duration > 4 months (P = 0.04), and WBC > 11 × 10(9) /L (P = 0.003) predicted inferior EFS; spine/abdomino-pelvic primary (P = 0.009) and WBC > 11 × 10(9) /L (P = 0.003) predicted inferior OS. Tumor size > 8 cm (P = 0.03) and radical radiotherapy as local treatment (P = 0.01) predicted inferior local-control-rate.
  
  
  CONCLUSIONS
  Prognostic hazard models for EFS and OS based on significant prognostic factors suggested that patients with combination of ESFT of spine/abdomino-pelvic region and baseline WBC > 11 × 10(9) /L had inferior OS (hazard ratio 4.44, P < 0.001) while patients with combination of ESFT with symptom duration > 4 months, tumor diameter > 8 m and baseline WBC > 11 × 10(9) /L had inferior EFS (hazard ratio 3.89, P = 0.002).
authors.affiliation: Department of Medical Oncology, Dr. B. R. Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Bivas
authors.initials: B
authors.lastname: Biswas
authors.forename: S
authors.initials: S
authors.lastname: Rastogi
authors.forename: S A
authors.initials: SA
authors.lastname: Khan
authors.forename: N K
authors.initials: NK
authors.lastname: Shukla
authors.forename: S V S
authors.initials: SV
authors.lastname: Deo
authors.forename: S
authors.initials: S
authors.lastname: Agarwala
authors.forename: B K
authors.initials: BK
authors.lastname: Mohanti
authors.forename: M C
authors.initials: MC
authors.lastname: Sharma
authors.forename: Sreenivas
authors.initials: S
authors.lastname: Vishnubhatla
authors.forename: S
authors.initials: S
authors.lastname: Bakhshi
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007958:Leukocyte Count; D008297:Male; D020360:Neoadjuvant Therapy; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012512:Sarcoma, Ewing / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D007069:Ifosfamide
keywords: chemotherapy; localized; outcome; primitive neuroectodermal tumor; prognostic factors