While multimodal therapy achieves approximately 70% survival in localized Ewing sarcoma, high relapse rates and poor outcomes in metastatic disease highlight the urgent need for novel biology-driven treatments.
Multimodal therapy remains standard for localized Ewing sarcoma; leaves open urgent need for novel biology-driven strategies to reduce relapse.
Ewing sarcoma, a rare malignancy of childhood and adolescence, has become a model of advances in diagnosis, treatment, and outcome through long-standing research efforts in multinational clinical trials. With modern multimodal regimens consisting of local surgery and/or radiotherapy plus intensive systemic chemotherapy, survival can be achieved for ≈ 70% of patients with localized disease. However, in the last decade, improvement in survival curves has slowed down. Also, a relapse rate of ≈ 30% remains unacceptable, since salvage strategies for Ewing sarcoma recurrence are discouraging and prognosis is unfavorable in most cases. Metastatic disease at diagnosis poses a similar challenge, since even if remission is achieved, relapse frequently occurs despite the most intensive treatment. Urgently needed, novel biology-driven treatment options are now beginning to emerge on the horizon, but have not yet reached the standard of care. An overview of the current clinical state-of-the-art is provided in this article.
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Potratz et al. (2012) studied this question.
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