期刊
JOURNAL OF CLINICAL ONCOLOGY
卷 33, 期 27, 页码 3036-U140出版社
AMER SOC CLINICAL ONCOLOGY
DOI: 10.1200/JCO.2014.59.5256
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类别
资金
- Unicancer
- la Societe Francaise des Cancers de l'Enfant
- Enfant et Sante
- German Cancer Aid [DKH 108128]
- Federal Ministry of Education and Research Germany (Translating Sarcoma Research Into Clinical Practice)
- Deutsches Zentrum fur Luft und Raumfahrt [01GM0869, 01ER0807]
- Euro-Ewing Consortium Grant [602856-2 EU FP7]
- PanCare-Life Grant [602030-2 EU-FP7]
- Prospektive Validierung von Biomarkern in Ewing Sarkomen European Research Area Network Translational Cancer Research Grant [01KT1310]
- European Network for Cancer Research in Children and Adolescents FP7-HEALTH-F2 Grant [261474]
- Swedish Childhood Cancer Foundation
- National Institute for Health Research University College London Hospitals Biomedical Research Centre
- Royal Marsden Hospital
- Cancer Research UK
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. (C) 2015 by American Society of Clinical Oncology
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