Portal de Periódicos da CAPES

Ewing Sarcoma: Current Management and Future Approaches Through Collaboration

2015; Lippincott Williams & Wilkins; Volume: 33; Issue: 27 Linguagem: Inglês

10.1200/jco.2014.59.5256

1527-7755

Nathalie Gaspar, Douglas S. Hawkins, Uta Dirksen, Ian Lewis, Stefano Ferrari, Marie‐Cécile Le Deley, Heinrich Kovar, R. J. Grimer, Jeremy Whelan, L. Claude, Olivier Delattre, Michael Paulussen, Piero Picci, Kirsten Sundby Hall, Hendrik Van den Berg, Ruth Ladenstein, Jean Michon, Lars Hjorth, Ian Judson, Roberto Luksch, Mark L. Bernstein, Perrine Marec‐Bérard, Bernadette Brennan, Alan Craft, Richard B. Womer, Heribert Juergens, Odile Oberlin,

Cardiac tumors and thrombi

Ewing sarcoma (ES) is an aggressive sarcoma of bone and soft tissue occurring at any age with a peak incidence in adolescents and young adults. The treatment of ES relies on a multidisciplinary approach, coupling risk-adapted intensive neoadjuvant and adjuvant chemotherapies with surgery and/or radiotherapy for control of the primary site and possible metastatic disease. The optimization of ES multimodality therapeutic strategies has resulted from the efforts of several national and international groups in Europe and North America and from cooperation between pediatric and medical oncologists. Successive first-line trials addressed the efficacy of various cyclic combinations of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin and identified prognostic factors now used to tailor therapies. The role of high-dose chemotherapy is still debated. Current 5-year overall survival for patients with localized disease is 65% to 75%. Patients with metastases have a 5-year overall survival < 30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrence have a dismal prognosis. The many insights into the biology of the EWS-FLI1 protein in the initiation and progression of ES remain to be translated into novel therapeutic strategies. Current options and future approaches will be discussed.