title: Ewing sarcoma: clinical state-of-the-art.
abstract: 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.
authors.affiliation: Pediatric Hematology and Oncology, University Children's Hospital Münster, Münster, Germany.
authors.forename: Jenny
authors.initials: J
authors.lastname: Potratz
authors.forename: Uta
authors.initials: U
authors.lastname: Dirksen
authors.forename: Heribert
authors.initials: H
authors.lastname: Jürgens
authors.forename: Alan
authors.initials: A
authors.lastname: Craft
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate