Treatment outcomes of extraskeletal Ewing sarcoma

J Surg Oncol. 2023 Jul;128(1):105-110. doi: 10.1002/jso.27250. Epub 2023 Mar 31.


Purpose: Extraskeletal Ewing sarcoma (EES), is a rare soft tissue sarcoma. Treatment for EES commonly involves chemotherapy and surgical resection (ST) or less commonly combined chemotherapy, surgery, and radiotherapy (ST + RT). The purpose of the current study was to evaluate our institutional experience treating EES.

Methods: We reviewed 36 (18 males:18 females) patients (mean age 30 years) with a nonretroperitoneal/visceral EES treated with either ST (n = 24, 67%) or ST + RT (n = 12, 33%). All patients were treated with chemotherapy, most commonly vincristine, doxorubicin, cyclophosphamide/ifosfamide and etoposide (VDC/IE, n = 23, 66%) Radiotherapy was mostly delivered preoperatively (n = 9). The mean follow-up was 8 years.

Results: The 10-year disease specific survival for patients was 78%, with no difference in the survival between patients in the ST versus the ST + RT groups (83% vs. 71%, p = 0.86). There was no difference in the 10-year local recurrence (91% vs. 100%, p = 0.29) or metastatic free survival (87% vs. 75%, p = 0.45) between the ST and ST + RT groups.

Conclusion: The results of the current study highlight the ability to achieve excellent local control with chemotherapy and surgery for EES. We recommend for multidisciplinary management of patients with EES, including chemotherapy and surgery, with use of radiotherapy if there is concern for a potentially close margin of resection.

Keywords: Ewing sarcoma; extraosseous; outcome; soft-tissue sarcoma.

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bone Neoplasms*
  • Cyclophosphamide
  • Doxorubicin
  • Etoposide / therapeutic use
  • Female
  • Humans
  • Male
  • Sarcoma* / drug therapy
  • Sarcoma, Ewing* / pathology
  • Sarcoma, Ewing* / therapy
  • Treatment Outcome
  • Vincristine / therapeutic use


  • Cyclophosphamide
  • Doxorubicin
  • Etoposide
  • Vincristine