This protocol addresses patients with Ewing's sarcoma who presented with pulmonary metastases at the time of initial diagnosis — a setting affecting approximately 10% of newly diagnosed cases. The presence of lung metastases at diagnosis marks a high-risk disease course and shapes both first- and subsequent-line management decisions.
The initial approach for this population is neoadjuvant induction chemotherapy with the interval-compressed VDC/IE regimen (vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide), followed by local therapy for the primary tumour and, where appropriate, whole-lung irradiation for responding lung metastases.
This protocol applies when that first-line course fails to achieve the following goals: complete histological tumour response on surgical specimen, adequate reduction in the soft tissue mass on MRI, or complete remission of all lung metastases.
After failure of the initial VDC/IE-based regimen, second-line treatment involves combination chemotherapy. Multiple evidence-based regimen options exist in this setting, each incorporating different classes of cytotoxic agents — the full structured protocol, including which options apply and how they are sequenced, is available via the link below.
DOI: 10.1016/j.annonc.2021.08.1995
Approximately 25% of patients are diagnosed with metastatic disease (10%: lung; 10%: bones/bone marrow; 5%: combinations or others).
Whole-lung irradiation, particularly when achieving complete remission of all lung metastases, can be used in this setting, although data demonstrating an improvement in outcome are lacking [III, C].
ChT regimens for relapsed ES are not standardised and include alkylating agents (cyclophosphamide and ifosfamide) in combination with topoisomerase inhibitors (etoposide and topotecan), irinotecan with temozolomide, gemcitabine and docetaxel, high-dose ifosfamide or carboplatin with etoposide [III, B].
Preliminary results from the rEECur study, the first randomised, controlled trial in this setting, suggest gemcitabine and docetaxel to be the inferior regimen, with temozolomide plus irinotecan also inferior to topotecan plus cyclophosphamide and high-dose ifosfamide.
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