This protocol addresses Ewing's sarcoma patients with pulmonary metastases whose disease was not progressing during initial induction chemotherapy, but for whom a subsequent line of chemotherapy has not achieved tumour response on imaging after four cycles.
The presence of pulmonary metastases with disease not progressing on induction chemotherapy identifies a distinct Ewing's sarcoma sub-population. This clinical status is central to the management considerations described in this protocol.
The preceding line — which may have included high dose ifosfamide, or cyclophosphamide and topotecan, or irinotecan and temozolomide — did not achieve the expected goal of tumour response on imaging after four cycles of chemotherapy. This failure of response is the trigger for escalation to the current protocol.
For patients with pulmonary metastases, whole lung irradiation (WLI) following VDC/IE chemotherapy is indicated if the disease is not progressing on induction chemotherapy.
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.
DOI: 10.1038/s41416-024-02868-4
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