title: Extraskeletal Ewing's sarcoma family of tumours in adults: analysis of 57 patients from a single institution.
abstract: OBJECTIVE
  Extraskeletal Ewing's sarcoma (EES) is a rare form of soft tissue sarcoma. The aim of the present study was to assess the outcome and the prognosis of adult patients presenting with EES treated with multi-modality therapy.
  
  
  METHODS
  All EES patients older than 15 years referred to our institution between January 1995 and December 2004 were reviewed. In total, 57 patients were identified. Their median age at diagnosis was 20 years (range 15-57).
  
  
  RESULTS
  The median size of the primary tumour was 11 cm (range 4-30 cm). Eighteen patients (31%) had metastatic disease at initial presentation. Wide surgical resection with negative margins was achieved in 23 cases (40%). Chemotherapy consisting of vincristine, adriamycin, ifosfamide, actinomycin D was given in 50 patients (88%). Radiotherapy was delivered in 37 patients (65%). Forty-one patients (72%) achieved complete remission and 16 (28%) progressed on therapy. Twenty-one patients (51%) relapsed. Local recurrence was encountered in 15 patients (36%). At a median follow-up of 46 months (range 6-143 months), the 5-year event-free survival and overall survival rates were 35 and 47%, respectively. Metastases at presentation, tumour size and surgical resection margin associated significantly with overall survival and event-free survival.
  
  
  CONCLUSIONS
  EES is an aggressive type of tumour with a high incidence of local recurrence and distant metastasis. This series showed that the outcome of adult EES is not unlike that of skeletal Ewing's sarcoma in terms of response to multi-modality treatment and the prognostic factors influencing treatment outcome. Adequate surgical resection, aggressive chemotherapy and adjuvant local radiation therapy, when indicated, constitute the optimal treatment to achieve the best results in this rare type of disease.
authors.affiliation: Department of Medical Oncology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: A
authors.initials: A
authors.lastname: El Weshi
authors.forename: A
authors.initials: A
authors.lastname: Allam
authors.forename: D
authors.initials: D
authors.lastname: Ajarim
authors.forename: F
authors.initials: F
authors.lastname: Al Dayel
authors.forename: R
authors.initials: R
authors.lastname: Pant
authors.forename: S
authors.initials: S
authors.lastname: Bazarbashi
authors.forename: M
authors.initials: M
authors.lastname: Memon
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D008875:Middle Aged; D011878:Radiotherapy; D012509:Sarcoma / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D012512:Sarcoma, Ewing / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D007069:Ifosfamide