TY - JOUR
T1 - Ewing Sarcoma: Current Management and Future Approaches Through Collaboration.
AU - Gaspar, Nathalie
AU - Hawkins, Douglas S
AU - Dirksen, Uta
AU - Lewis, Ian J
AU - Ferrari, Stefano
AU - Le Deley, Marie-Cecile
AU - Kovar, Heinrich
AU - Grimer, Robert
AU - Whelan, Jeremy
AU - Claude, Line
AU - Delattre, Olivier
AU - Paulussen, Michael
AU - Picci, Piero
AU - Sundby Hall, Kirsten
AU - van den Berg, Hendrik
AU - Ladenstein, Ruth
AU - Michon, Jean
AU - Hjorth, Lars
AU - Judson, Ian
AU - Luksch, Roberto
AU - Bernstein, Mark L
AU - Marec-Bérard, Perrine
AU - Brennan, Bernadette
AU - Craft, Alan W
AU - Womer, Richard B
AU - Juergens, Heribert
AU - Oberlin, Odile
PY - 2015
Y1 - 2015
N2 - 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.
AB - 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.
U2 - 10.1200/JCO.2014.59.5256
DO - 10.1200/JCO.2014.59.5256
M3 - Review article
C2 - 26304893
SN - 1527-7755
VL - 33
SP - 3036-U140
JO - Journal of Clinical Oncology
JF - Journal of Clinical Oncology
IS - 27
ER -