How to Treat the Ewing’s Family of Sarcomas in Adult Patien.docVIP

How to Treat the Ewing’s Family of Sarcomas in Adult Patien 　 【关键词】 Ewings,sarcoma,amp;#8226;,Adult,Prognosis,amp;#8226;,Survival 　　LEARNING OBJECTIVES 　　After completing this course, the reader will be able to: 　　Describe how ESFT is not only a disease of children, but equally that of young adults. 　　Discuss the established prognostic variables in ESFT and the unresolved controversy surrounding the role of older age in patient outcome. 　　Explain that until large studies, such as the current EuroEwings study, can prospectively define the role of age as a prognostic variable, adult patients with ESFT need to be treated as intensively as younger patients. 　　ABSTRACT 　　Ewings sarcoma, peripheral primitive neuroectodermal tumor, and Askin?s tumor comprise a single family of tumors, the Ewing?s family of tumors, which is characterized by chromosomal translocation. Ewing?s sarcoma is known as a malignancy of childhood, but with a median age of 15 years at diagnosis, it should equally be regarded as a malignancy of adolescence and young adulthood. There is much controversy regarding the role of age at diagnosis, with some studies showing older age to be associated with poorer outcome and others showing no association between age and survival. This has led to uncertainty in how best to manage nonpediatric patients with Ewing’s sarcoma. This article examines whether age does affect outcome and treatment in this group of tumors. 　　INTRODUCTION 　　In 1921, James Ewing described the case of a 14-year-old girl with a highly malignant bone tumor composed of small round cells and named the condition diffuse endothelioma [1]. It is now known as Ewing?s sarcoma (ES). It is recognized that ES is part of a family called the Ewing?s sarcoma family of tumors (ESFT), which also includes peripheral primitive neuroectodermal tumor (PNET), extraosseous Ewing?s sarcoma (EES), and Askin?s tumor (ES of the chest wall). 　　ES is regarded as a malignancy of childhood and adolescence. I