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Management of recurrent Ewing sarcoma: challenges and approaches

Authors Van Mater D, Wagner L

Received 4 December 2018

Accepted for publication 15 February 2019

Published 27 March 2019 Volume 2019:12 Pages 2279—2288

DOI https://doi.org/10.2147/OTT.S170585

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 4

Editor who approved publication: Dr Carlos E Vigil


David Van Mater, Lars Wagner

Department of Pediatrics, Division of Hematology/Oncology, Duke University, Durham, NC, USA

Abstract: Although many patients with newly diagnosed Ewing sarcoma can become long-term survivors, relapse remains an important clinical problem for which there is no standard approach. Several prognostic factors have been identified, and these may help guide patient counseling and therapy decisions. A variety of chemotherapy regimens have produced responses in patients with recurrent Ewing sarcoma, but no comparative studies have been completed to show superiority of any one particular approach. In addition, the optimum length of therapy for salvage regimens and use of local control measures remains unknown. The likelihood of cure remains low and the gaps in our knowledge are great, and so enrollment on clinical trials should be strongly encouraged for these patients when feasible. Because Ewing sarcoma is relatively rare, some pediatric and adult oncologists may be less familiar with the management of relapsed patients. In this review, we address common questions facing the clinician and patient, and provide an update on new strategies for therapy.

Keywords: relapsed Ewing sarcoma, adolescent and young adult oncology, AYA, irinotecan, topotecan

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