Seeking international consensus on approaches to primary tumour treatment in Ewing sarcoma
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Clinical Sarcoma Research Open Access
RESEARCH
Seeking international consensus on approaches to primary tumour treatment in Ewing sarcoma Craig Gerrand1*, Jessica Bate2, Beatrice Seddon3, Uta Dirksen4, R. Lor Randall5, Michiel van de Sande6 , Paul O’Donnell1, John Tuckett7, David Peake8, Lee Jeys9 , Asif Saifuddin1, Mel Grainger10 and Jeremy Whelan3
Abstract Background: The local treatment of Ewing sarcoma of bone involves surgery, radiotherapy or both. The selection of treatment depends on the anatomical extent of the tumour, the effectiveness of the proposed treatment, its morbidity, and the expectation of cure. However, not only are there variations in the approach to local treatment between individual patients, but also between treatment centres and countries. Our aim was to explore variation in practice and develop consensus statements about local treatment. Methods: A three stage modified Delphi technique was used with international collaborators. This involved an expert panel to identify areas of controversy, an online survey of international collaborators and a consensus meeting in London, UK in June 2017. In the consensus meeting, teams of clinicians discussed the local management of selected cases and their responses were collected with electronic voting. Results: Areas of greater or less consensus were identified. The lack of evidence underpinning different approaches was noted and areas for collaborative research became apparent. Conclusion: This has demonstrated that there is an international consensus around many aspects of the local treatment of Ewing sarcoma of bone, including the use of specialist MultiDisciplinary Team (MDT) meetings with access to all appropriate treatments. However, considerable variation remains including the use of different staging investigations, decision making, definitions of response, and radiotherapy doses and timing. Further collaborative work should be undertaken to determine the impact of these variations in order to define best practice. Keywords: Ewing sarcoma, Surgery, Radiotherapy, Limb salvage, Combined modality Background Ewing sarcoma is the second most common primary bone tumour occurring in children and young adults, with an incidence of approximately 1 per million. It typically occurs in the second and third decades of life, with
*Correspondence: [email protected] 1 Royal National Orthopaedic Hospital, Brockley Hill, Stanmore HA7 4LP, Middlesex, UK Full list of author information is available at the end of the article
80% of patients under 20 years of age at diagnosis [1]. Ewing sarcoma can occur in any bone, with a distribution reflecting the mass of bone in the skeleton [2]. Ewing sarcoma of bone classically presents with pain and an extra-osseous mass arising from the affected bone. Characteristic imaging features include a periosteal reaction with or without bone destruction on plain radiographs, and an extra-osseous mass on magnetic resonance imaging (MRI). Most cases demonstrate a translocation involving chromosomes 11 and 22 [3].
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