TY - CHAP M1 - Book, Section TI - Chapter 454. Ewing Sarcoma and Primitive Neuroectodermal Tumor A1 - Mackall, Crystal L. A1 - Toretsky, Jeffrey A. A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. Y1 - 2011 N1 - T2 - Rudolph's Pediatrics, 22e AB - Ewing sarcoma (ES) is the second most common bone tumor in children and adolescents. It was named for James Ewing, a pathologist who first described the tumor in 1921, emphasizing its distinction from osteosarcoma based upon enhanced radiosensitivity and a propensity to involve flat bones of the axial skeleton.1 In the early 1980s, both Ewing sarcoma and peripheral primitive neuroectodermal tumor (PNET) were found to contain identical t(11;22)(q24;q12) translocations.2,3 Based upon the shared translocation, and similar clinical behaviors, Ewing Sarcoma Family of Tumors (ESFT) is now considered one disease entity that includes classical ES, atypical ES, peripheral primitive neuroectodermal tumor, neuroepithelioma, and Askin tumor (an ESFT of the chest wall). It should be emphasized that peripheral PNETs are entirely different from PNETs arising in the central nervous system which do not bear the t(11;22) translocation and are not considered part of the ESFT category. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/25 UR - accesspediatrics.mhmedical.com/content.aspx?aid=7043301 ER -