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Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Orthop. Sep 18, 2016; 7(9): 527-538
Published online Sep 18, 2016. doi: 10.5312/wjo.v7.i9.527
Management of Ewing sarcoma family of tumors: Current scenario and unmet need
Bivas Biswas, Sameer Bakhshi
Bivas Biswas, Sameer Bakhshi, Department of Medical Oncology, Dr.B.R.A.I.R.C.H, All India Institute of Medical Sciences, New Delhi 110029, India
Author contributions: Biswas B performed the literature search, critical analysis, wrote the paper recorded the audio core tip; Bakhshi S reviewed and edited the paper and done the critical analysis.
Conflict-of-interest statement: No potential conflicts of interest to declare for each author.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Dr. Bivas Biswas, Assistant Professor, Department of Medical Oncology, Dr.B.R.A.I.R.C.H, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. bivasbiswas@gmail.com
Telephone: +91-98-30922005 Fax: +91-11-26588663
Received: April 27, 2016
Peer-review started: April 28, 2016
First decision: June 16, 2016
Revised: June 21, 2016
Accepted: July 11, 2016
Article in press: July 13, 2016
Published online: September 18, 2016
Processing time: 137 Days and 10 Hours
Abstract

Ewing sarcoma family tumors (ESFT) are heterogeneous, aggressive group of disease with peak incidence in adolescent and young adults. The outcome has been improved dramatically from 10% with surgery and radiotherapy alone to 65%-70% now, in localized disease, with the introduction of chemotherapy. Chemotherapy regimen evolved from single agent to multiagent with effort of many cooperative clinical trials over decades. The usual treatment protocol include introduction of multi-agent chemotherapy in neoadjuvant setting to eradicate systemic disease with timely incorporation of surgery and/or radiotherapy as local treatment modality and further adjuvant chemotherapy to prevent recurrence. Risk adapted chemotherapy in neoadjuvant and adjuvant setting along with radiotherapy has been used in many international collaborative trials and has resulted in improved outcome, more so in patients with localized disease. The role of high dose chemotherapy with stem cell rescue is still debatable. The outcome of patients with metastatic disease is dismal with long term outcome ranges from 20%-40% depending on the sites of metastasis and intensity of treatment. There is a huge unmet need to improve outcome further, more so in metastatic setting. Novel therapy targeting the molecular pathways and pathogenesis of ESFT is very much required. Here we have discussed the current standard of management in patients with ESFT, investigational targeted or novel therapies along with future promises.

Keywords: Outcome; Review; Chemotherapy; Ewing sarcoma; Targeted therapy; Radiotherapy; Prognostic factors

Core tip: Ewing sarcoma family tumors are a heterogeneous and aggressive group of disease of bone and soft tissue in childhood. The outcome has improved with introduction of chemotherapy and multimodality management. But, the prognosis of patients with metastatic disease is dismal. Novel targeted therapies are investigational and may offer some hope in future, especially in metastatic setting. In this review we have discussed current treatment modality, prognostic factors, ongoing trials and novel investigational therapies.