TY - CHAP M1 - Book, Section TI - Ewing Sarcoma and Primitive Neuroectodermal Tumors A1 - DuBois, Steven G. A1 - Yustein, Jason T. A2 - Kline, Mark W. PY - 2018 T2 - Rudolph's Pediatrics, 23e AB - Ewing sarcoma was first described in 1921 by James Ewing as a radiosensitive malignancy of the bone. Since that initial description, insights into the biology of this tumor have expanded the definition of this disease. Based upon our current understanding, Ewing sarcoma comprises a family of tumors that share a common set of chromosomal translocations that are believed to be the originating event driving the pathogenesis of these tumors (see below). Ewing sarcomas may arise in the bone or soft tissues throughout the body. They may be undifferentiated (pathologically termed Ewing sarcoma) or show evidence of neural differentiation (pathologically termed primitive neuroectodermal tumor, or PNET). Moreover, a subset of these tumors that arise in the chest wall have historically been referred to as Askin tumors. As Ewing sarcoma, PNET, and Askin tumors are now understood to share the same fundamental biology, the principles that drive the management of patients with these tumors are largely identical. Therefore, except where relevant differences exist, this chapter will refer to these tumors simply as Ewing sarcoma. This is in contradistinction to a more recently recognized group of tumors that have been classified as Ewing-like sarcomas. These tumors harbor different genetic translocations and are biologically distinct from Ewing sarcoma, and little is known about their optimal management. Further discussion of Ewing-like sarcomas is outside the scope of this chapter. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/11 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1182911351 ER -