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Establishing long-term survival and cure in young patients with Ewing's sarcoma

Lookup NU author(s): Emeritus Professor Alan Craft


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This paper investigates the potential for long-term survivorship for young patients diagnosed with Ewing's sarcoma. Data are examined from two successive UKCCSG Ewing's Tumour studies (ET-1 and ET-2). Patients have been followed for up to 20 years. These studies had suggested that better 5-year survival with ET-2 over the earlier ET-1 was achieved by replacing cyclophosphamide by ifosfamide and increasing the dose of doxorubicin in a four-drug chemotherapy regimen. The updated hazard ratio, stratified for metastatic status at diagnosis, of 0.39 (95% confidence interval 0.12-0.61) confirmed the advantage of the ET-2 regimen in terms of overall survival. Cure models, based on the Weibull distribution, suggested that factors for long-term survival in addition to presence of metastases were age, primary site of tumour and study. Modelling identified the proportion cured with the ET-2 protocol as best at 70% in those who are under 10 years with a nonpelvic primary site and without metastatic disease. This contrasts to only 13% cure in those with the corresponding adverse prognostic indicators. Additionally, the risk of death remains greatest but relatively constant over the first 2 years postdiagnosis, and then declines to a lower but constant value for the next 3 years before reaching the 'cure plateau' at about 5 years. This investigation suggests that 'cure' is possible for patients with Ewing's sarcoma. This is established at approximately 5 years post diagnosis and the proportion cured depends on the presence of metastases, pelvic site and age at diagnosis.

Publication metadata

Author(s): Weston, C. L., Douglas, C., Craft, A. W., Lewis, I. K., Machin, D.

Publication type: Article

Publication status: Published

Journal: British Journal of Cancer

Year: 2004

Volume: 91

Issue: 2

Pages: 225-232

ISSN (print): 0007-0920

ISSN (electronic): 1532-1827


DOI: 10.1038/sj.bjc.6601955

PubMed id: 15213720

Notes: Group Authors: UKCCSG


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