Treatment of Recurrent Ewing Sarcoma

Source: Ewing Sarcoma and Undifferentiated Small Round Cell Sarcomas of Bone and Soft Tissue Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: November 27, 2024 · Captured 2026-09-09.

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Recurrence of Ewing sarcoma is most common within 2 years of initial diagnosis (approximately 80%).[1,2] However, late relapses occurring more than 5 years from initial diagnosis are more common in Ewing sarcoma (13%; 95% confidence interval, 9.4%–16.5%) than in other pediatric solid tumors.[3] An analysis of the Surveillance, Epidemiology, and End Results (SEER) Program database identified 1,351 patients who survived more than 60 months from diagnosis.[4] Of these patients, 209 died; 144 of the deaths (69%) were attributed to recurrent, progressive Ewing sarcoma. Black race, male sex, older age at initial diagnosis, and primary tumors of the pelvis and axial skeleton were associated with a higher risk of late death. This analysis covered the period from 1973 to 2013, and the 1,351 patients represented only 38% of the patients in the original sample, which reflects the inferior treatment outcomes from the earlier era. It is possible that patients who reach the 5-year point after more contemporary treatment may not recapitulate this experience.

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The overall prognosis for patients with recurrent Ewing sarcoma is poor. The 5-year survival rate after recurrence is approximately 10% to 15%.[2,5,6]; [1][Level of evidence C1] Patients with relapsed or progressive Ewing sarcoma with measurable disease have a 6-month event-free survival (EFS) rate of 13%.[7][Level of evidence C1]

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Prognostic factors include the following:

Time to recurrence. Time to recurrence is the most important prognostic factor. Patients whose Ewing sarcoma recurred more than 2 years from initial diagnosis had a 5-year survival rate of 30%, versus 7% for patients whose disease recurred within 2 years.[1,2]

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Local and distant recurrence. Patients with both local recurrence and distant metastases have a worse outcome than patients with either isolated local recurrence or metastatic recurrence alone.[1,2]

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Isolated pulmonary recurrence. Isolated pulmonary recurrence was not an important prognostic factor in a North American series.[1] In the Italian/Scandinavian experience, younger age, longer disease-free interval, and lung-only recurrence were associated with longer progression-free survival (PFS) after recurrence. In this experience, patients with Ewing sarcoma that recurred after initial therapy, which included high-dose therapy with autologous stem cell rescue, were less likely to achieve a second complete remission.[8][Level of evidence C2]

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The selection of treatment for patients with recurrent disease depends on many factors, including the following:

Site of recurrence.

Previous treatment.

Individual patient considerations.

There is no standardized second-line treatment for patients with relapsed or refractory Ewing sarcoma. Most patients in first relapse are treated with conventional systemic chemotherapy. Patients who demonstrate a response to therapy may undergo local control to sites of recurrence.

Treatment options for recurrent Ewing sarcoma include the following:

Chemotherapy.

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