EWING SARCOMA TREATMENT

J Clin Oncol. 2015 Aug 24. pii: JCO.2014.59.5256. [Epub ahead of print]

Ewing Sarcoma: Current Management and Future Approaches Through Collaboration.

Gaspar N1, Hawkins DS2, Dirksen U2, Lewis IJ2, Ferrari S2, Le Deley MC2, Kovar H2, Grimer R2, Whelan J2, Claude L2, Delattre O2, Paulussen M2, Picci P2, Sundby Hall K2, van den Berg H2, Ladenstein R2, Michon J2, Hjorth L2, Judson I2, Luksch R2, Bernstein ML2, Marec-Bérard P2, Brennan B2, Craft AW2, Womer RB2, Juergens H2, Oberlin O2.

Author information

Abstract

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.