Ewing sarcoma: clinical state-of-the-art

Pediatr Hematol Oncol. 2012 Feb;29(1):1-11. doi: 10.3109/08880018.2011.622034.


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.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Humans
  • Male
  • Neoplasm Metastasis
  • Sarcoma, Ewing / diagnosis*
  • Sarcoma, Ewing / mortality
  • Sarcoma, Ewing / pathology
  • Sarcoma, Ewing / therapy*
  • Survival Rate