Originally published as JCO Early Release 10.1200/JCO.2005.05.105 on March 21 2005

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Survival After Recurrence of Ewing’s Sarcoma Family of Tumors

From the University of Washington School of Medicine; Children’s Hospital and Regional Medical Center; and Fred Hutchinson Cancer Research Center, Seattle, WA

Address reprint requests to Douglas Hawkins, MD, Children’s Hospital and Regional Medical Center, 4800 Sandpoint Way NE, MS: 6D-1, Seattle, WA 98105-0371; e-mail: Doug.Hawkins{at}seattlechildrens.org

PURPOSE: The overall survival (OS) of patients with relapsed Ewing’s sarcoma family of tumors (ESFT) is poor, and the relative benefit of high-dose therapy (HDT) is controversial.

PATIENTS AND METHODS: We retrospectively identified 55 consecutive ESFT patients with adequate medical records for review, who were treated at Children’s Hospital and Regional Medical Center and who developed disease recurrence between January 1, 1985 and December 31, 2002.

RESULTS: The median relapse-free interval (RFI) from diagnosis to first recurrence was 17 months (range, 5 to 90 months). Most recurrences were metastatic only (39 patients) or local and metastatic (10 patients). Twenty-seven patients (49%) achieved a partial or complete response to second-line treatment, with a median duration of response of 27 months (range, 5 to 119+ months). The 5-year OS rate for all relapsed patients was 23% (95% CI, 11% to 35%). By univariate analysis, improved OS was associated with response to second-line treatment versus no response (46% v 0%, respectively; P < .0001), RFI 24 months versus less than 24 months (48% v 12%, respectively; P = .0001), and no metastases at initial diagnosis versus presence of metastases (31% v 12%, respectively; P = .05). Because all 13 patients who received HDT also had responsive relapse, we performed a multivariate analysis. Reduced risk of death was associated with response to second-line therapy (relative risk, 0.14; 95% CI, 0.05 to 0.40), RFI 24 months (relative risk, 0.29; 95% CI, 0.13 to 0.66), and receiving HDT (relative risk, 0.26; 95% CI, 0.08 to 0.85).

CONCLUSION: HDT as consolidation therapy for relapsed ESFT seems to be associated with improved OS, even after adjusting for RFI and response to second-line treatment.

Supported in part by the Rex and Arlene Garrison Student Summer Fellowships from the American Cancer Society and National Institutes of Health grant No. CA-87721.

Presented in abstract form at the 40th Annual Meeting of the American Society of Clinical Oncology, New Orleans, LA, June 5-8, 2004.

Authors’ disclosures of potential conflicts of interest are found at the end of this article.

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