Highlights
- •Ewing sarcoma is a malignant bone tumour with a predilection for young people.
- •Refractory or recurrent disease has a dismal prognosis.
- •High-dose chemotherapy and autologous stem-cell transplant may prolong survival.
- •Risk stratification at relapse may aid clinical decision-making.
Abstract
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
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Article info
Publication history
Published online: May 26, 2022
Accepted:
April 1,
2022
Received in revised form:
March 29,
2022
Received:
February 16,
2022
Identification
Copyright
Crown Copyright © 2022 Published by Elsevier Ltd. All rights reserved.
