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Ewing Sarcoma
Andrew T. Trout, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Ewing sarcoma (ES) family of tumors
                • Osseous ES
                • Extraosseous ES
                • Askin tumor
                • Primitive neuroectodermal tumor (PNET)
              • Named after James Ewing, cofounder of American Cancer Society
              • Malignant small round cell tumor
              • Majority have translocation between EWSR1 (chromosome 22) and ETS transcription factor

            IMAGING

            • General Features

              • MR Findings

                • F-18 FDG PET/CT Findings

                  • F-18 FLT PET Findings

                    • PET/MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              Selected References

                                              1. Koshkin VS et al: Assessment of imaging modalities and response metrics in ewing sarcoma: correlation with survival. J Clin Oncol. ePub, 2016
                                              2. Osgood CL et al: 18F-FLT Positron Emission Tomography (PET) is a Pharmacodynamic Marker for EWS-FLI1 Activity and Ewing Sarcoma. Sci Rep. 6:33926, 2016
                                              3. Choi EY et al: Ewing sarcoma. Semin Diagn Pathol. 31(1):39-47, 2014
                                              4. Cote GM et al: Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin North Am. 27(5):1007-19, 2013
                                              5. Grohar PJ et al: Prospects and challenges for the development of new therapies for Ewing sarcoma. Pharmacol Ther. 137(2):216-24, 2013
                                              6. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                              7. Ross KA et al: The biology of ewing sarcoma. ISRN Oncol. 2013:759725, 2013
                                              8. Treglia G et al: Diagnostic accuracy of ¹⁸F-FDG-PET and PET/CT in patients with Ewing sarcoma family tumours: a systematic review and a meta-analysis. Skeletal Radiol. 41(3):249-56, 2012
                                              9. Eary JF et al: Imaging in sarcoma. J Nucl Med. 52(12):1903-13, 2011
                                              10. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                              11. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                              12. Ludwig JA: Ewing sarcoma: historical perspectives, current state-of-the-art, and opportunities for targeted therapy in the future. Curr Opin Oncol. 20(4):412-8, 2008
                                              13. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                              14. Gerth HU et al: Significant benefit of multimodal imaging: PET/CT compared with PET alone in staging and follow-up of patients with Ewing tumors. J Nucl Med. 48(12):1932-9, 2007
                                              15. Riggi N et al: The Biology of Ewing sarcoma. Cancer Lett. 254(1):1-10, 2007
                                              16. Bernstein M et al: Ewing's sarcoma family of tumors: current management. Oncologist. 11(5):503-19, 2006
                                              17. Gyorke T et al: Impact of FDG PET for staging of Ewing sarcomas and primitive neuroectodermal tumours. Nucl Med Commun. 27(1):17-24, 2006
                                              18. Alfeeli MA et al: Ewing sarcoma of the rib with normal blood flow and blood pool imagings on a 3-phase bone scan. Clin Nucl Med. 30(9):610-1, 2005
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Definitions

                                                          • Ewing sarcoma (ES) family of tumors
                                                            • Osseous ES
                                                            • Extraosseous ES
                                                            • Askin tumor
                                                            • Primitive neuroectodermal tumor (PNET)
                                                          • Named after James Ewing, cofounder of American Cancer Society
                                                          • Malignant small round cell tumor
                                                          • Majority have translocation between EWSR1 (chromosome 22) and ETS transcription factor

                                                        IMAGING

                                                        • General Features

                                                          • MR Findings

                                                            • F-18 FDG PET/CT Findings

                                                              • F-18 FLT PET Findings

                                                                • PET/MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          Selected References

                                                                                          1. Koshkin VS et al: Assessment of imaging modalities and response metrics in ewing sarcoma: correlation with survival. J Clin Oncol. ePub, 2016
                                                                                          2. Osgood CL et al: 18F-FLT Positron Emission Tomography (PET) is a Pharmacodynamic Marker for EWS-FLI1 Activity and Ewing Sarcoma. Sci Rep. 6:33926, 2016
                                                                                          3. Choi EY et al: Ewing sarcoma. Semin Diagn Pathol. 31(1):39-47, 2014
                                                                                          4. Cote GM et al: Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin North Am. 27(5):1007-19, 2013
                                                                                          5. Grohar PJ et al: Prospects and challenges for the development of new therapies for Ewing sarcoma. Pharmacol Ther. 137(2):216-24, 2013
                                                                                          6. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                                                                          7. Ross KA et al: The biology of ewing sarcoma. ISRN Oncol. 2013:759725, 2013
                                                                                          8. Treglia G et al: Diagnostic accuracy of ¹⁸F-FDG-PET and PET/CT in patients with Ewing sarcoma family tumours: a systematic review and a meta-analysis. Skeletal Radiol. 41(3):249-56, 2012
                                                                                          9. Eary JF et al: Imaging in sarcoma. J Nucl Med. 52(12):1903-13, 2011
                                                                                          10. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                                                                          11. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                                                                          12. Ludwig JA: Ewing sarcoma: historical perspectives, current state-of-the-art, and opportunities for targeted therapy in the future. Curr Opin Oncol. 20(4):412-8, 2008
                                                                                          13. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                                                                          14. Gerth HU et al: Significant benefit of multimodal imaging: PET/CT compared with PET alone in staging and follow-up of patients with Ewing tumors. J Nucl Med. 48(12):1932-9, 2007
                                                                                          15. Riggi N et al: The Biology of Ewing sarcoma. Cancer Lett. 254(1):1-10, 2007
                                                                                          16. Bernstein M et al: Ewing's sarcoma family of tumors: current management. Oncologist. 11(5):503-19, 2006
                                                                                          17. Gyorke T et al: Impact of FDG PET for staging of Ewing sarcomas and primitive neuroectodermal tumours. Nucl Med Commun. 27(1):17-24, 2006
                                                                                          18. Alfeeli MA et al: Ewing sarcoma of the rib with normal blood flow and blood pool imagings on a 3-phase bone scan. Clin Nucl Med. 30(9):610-1, 2005