title: Extraskeletal Ewing's sarcoma family of tumors in adults: prognostic factors and clinical outcome.
abstract: OBJECTIVE
  The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with extraskeletal Ewing's sarcoma.
  
  
  METHODS
  Data of patients with extraskeletal Ewing's sarcoma followed up at our center between 1997 and 2010 were retrospectively analyzed.
  
  
  RESULTS
  The median age of 27 patients was 24 years (range, 16-54 years). The median follow-up was 31.8 months (range, 6-144 months). Tumor size was between 1.5 and 14 cm (median: 8 cm). Eighty-five percent of patients had localized disease at presentation and 15% had metastatic disease. Local therapy was surgery alone in 16% of patients, surgery combined with radiotherapy in 42% and radiotherapy alone in 27%. All patients were treated with vincristine, doxorubicin, cyclophosphamide and actinomycin-D, alternating with ifosfamide and etoposide every 3 weeks. In patients with localized disease at presentation, the 5-year event-free survival and overall survival were 59.7 and 64.5%, respectively. At univariate analysis, patients with tumor size ≥ 8 cm, high serum lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy and positive surgical margin had significantly worse event-free survival. The significant predictors of worse overall survival at univariate analysis were tumor size 8 ≥ cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin.
  
  
  CONCLUSIONS
  Prognostic factors were similar to primary osseous Ewing's sarcomas. Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewing's sarcoma.
authors.affiliation: Department of Medical Oncology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey. deniztural@gmail.com
authors.forename: Deniz
authors.initials: D
authors.lastname: Tural
authors.forename: Nil
authors.initials: N
authors.lastname: Molinas Mandel
authors.forename: Sergulen
authors.initials: S
authors.lastname: Dervisoglu
authors.forename: Fazilet
authors.initials: F
authors.lastname: Oner Dincbas
authors.forename: Sedat
authors.initials: S
authors.lastname: Koca
authors.forename: Didem
authors.initials: D
authors.lastname: Colpan Oksuz
authors.forename: Fatih
authors.initials: F
authors.lastname: Kantarci
authors.forename: Hande
authors.initials: H
authors.lastname: Turna
authors.forename: Fatih
authors.initials: F
authors.lastname: Selcukbiricik
authors.forename: Murat
authors.initials: M
authors.lastname: Hiz
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000097:blood; D017024:Chemotherapy, Adjuvant; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D015994:Incidence; D007770:L-Lactate Dehydrogenase / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis; D018365:Neoplasm, Residual / Q000175:diagnosis; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012307:Risk Factors; D012512:Sarcoma, Ewing / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014408:Biomarkers, Tumor; D003609:Dactinomycin; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D007770:L-Lactate Dehydrogenase; D007069:Ifosfamide