Ewing's Sarcoma
ICD-10 C40 · ICD-11 2B52

Ewing's Sarcoma After Failure to Achieve Tumour Response with Prior Second-Line Chemotherapy

This protocol applies when Ewing's sarcoma has not responded to a prior second-line chemotherapy regimen — specifically when imaging did not demonstrate the required tumour response after four cycles of treatment.

The preceding line of therapy — which may have included high-dose ifosfamide, or combinations such as cyclophosphamide and topotecan, or irinotecan and temozolomide, with or without high-dose chemotherapy consolidation with peripheral blood stem cell rescue — did not achieve tumour response on imaging by the four-cycle assessment point.

Failure criterion: no tumour response on imaging after four cycles

Options at this stage include multi-targeted tyrosine kinase inhibitors and a range of further cytotoxic approaches; the full selection criteria, sequencing, and individualisation are detailed in the structured regimen.

Complete regimen, agent selection, and sequencing available below.

References

DOI: 10.1038/s41416-024-02868-4

  • The most promising in terms of activity, toxicity and availability are the multi-targeted tyrosine kinase inhibitors.
  • Pazopanib, cabozantinib and regorafenib have been reported to show single-agent activity.
  • Oral etoposide is also frequently used to treat recurrent Ewing sarcoma.
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