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Ewing Sarcoma



Treatment Modality


The treatment of Ewing sarcoma (ES) requires a combined modality approach. 

Therapy must be multidisciplinary with a team of surgical, medical and radiation oncologists.

Chemotherapy is critical to have any hope of achieving local or distant control.  

Local control also requires:

  • Surgery
  • Radiation therapy (RT)
  • Combination of both surgery and RT.


Multidrug chemotherapy for ES includes:


The current COG study is AEWS1031

This is a phase III randomized trial of: Adding Vincristine-topotecan-cyclophosphamide to standard chemotherapy in initial treatment of non-metastatic Ewing sarcoma.

The intergroup Ewing sarcoma study INT-0091 (CCG 7881, POG 8850) showed that a regimen of alternating vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide was superior to vincristine-doxorubicin-cyclophosphamide alone.

AEWS0031 demonstrated interval compression to be superior to standard chemotherapy timing.

The five drug alternating combination using interval compression should be considered the North American standard therapy for localized Ewing sarcoma.

Cyclophosphamide with topotecan has been shown to be active in patients with recurrent and metastatic Ewing sarcoma.

COG AEWS1031 randomized Phase 3 trial will test the efficacy of adding vincristine, topotecan and cyclophosphamide to the interval compressed 5 drug backbone.

This study will assess initial tumor volume, histologic response to induction chemotherapy and response measured by FDG-PET as prognostic factors for event free survival in patients with non-metastatic Ewing sarcoma.


External Link:

Ewing treatment option overview at the National Cancer Institute










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