Survival after high-dose chemotherapy for refractory and recurrent Ewing sarcoma

Eur J Cancer. 2022 Jul:170:131-139. doi: 10.1016/j.ejca.2022.04.007. Epub 2022 May 26.


Background: outcome of primary refractory or recurrent Ewing sarcoma (RRES) is poor and the role of high-dose therapy (HDT) remains uncertain. We retrospectively reviewed all patients treated for RRES in the London Sarcoma Service (LSS) over a 22-year period with the aim of adding to the current literature and developing a prognostic risk score to aid clinical decision-making.

Methods and results: One hundred and ninety-six patients were included; 64 patients received HDT, 98 standard non-HDT chemotherapy and 34 no systemic therapy. At RRES, median age was 20 years and seventy-four per cent of patients had progressed or relapsed within 24 months. Median overall survival for HDT and non-HDT patients was 76 months (95% CI 34.8-117.2) and 10.5 months (95% CI 8.9-12.1), respectively. Two and five-year post-relapse survival (PRS) for HDT patients was 67.9% (SE 5.9) and 52.7% (SE 6.5), and for non-HDT patients, 20.5% (SE 4.2) and 2% (SE 1.5). Four prognostic factors significant on multivariate analysis were assigned a score of one point each, creating good (score 0), intermediate (score 1-2) and poor (score 3-4) prognosis groups. The increased score was significantly associated with reduced PRS.

Conclusion: Our study demonstrates that in RRES, HDT is associated with superior outcomes compared with non-HDT chemotherapy. RRES patients can be risk-stratified according to a predictive prognostic index we have developed, with the potential benefit of HDT observed even in patients with poor prognostic scores.

Keywords: Autologous stem-cell transplant; Ewing; Sarcoma.

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bone Neoplasms
  • Combined Modality Therapy
  • Disease-Free Survival
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Neoplasm Recurrence, Local / drug therapy
  • Prognosis
  • Retrospective Studies
  • Sarcoma, Ewing* / drug therapy
  • Transplantation, Autologous
  • Treatment Outcome
  • Young Adult

Supplementary concepts

  • Askin Tumor