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Ewing Sarcoma
Andrew T. Trout, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Ewing sarcoma (ES)
          • Definitions

            • 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

            • 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. Huang T et al: Effectiveness of 18F-FDG PET/CT in the diagnosis, staging and recurrence monitoring of Ewing sarcoma family of tumors: A meta-analysis of 23 studies. Medicine (Baltimore). 97(48):e13457, 2018
                                    2. Ruggiero A et al: Diagnostic accuracy of 18F-FDG PET/CT in the staging and assessment of response to chemotherapy in children with Ewing sarcoma. J Pediatr Hematol Oncol. 40(4):277-84, 2018
                                    3. Palmerini E et al: The role of FDG PET/CT in patients treated with neoadjuvant chemotherapy for localized bone sarcomas. Eur J Nucl Med Mol Imaging. 44(2):215-23, 2017
                                    4. Hwang JP et al: Prognostic value of SUVmax measured by pretreatment fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Ewing sarcoma. PLoS One. 11(4):e0153281, 2016
                                    5. Raciborska A et al: Response to chemotherapy estimates by FDG PET is an important prognostic factor in patients with Ewing sarcoma. Clin Transl Oncol. 18(2):189-95, 2016
                                    6. Gelfand MJ et al: [F-18.] Pediatr Radiol. 45(8):1182-8, 2015
                                    7. Choi EY et al: Ewing sarcoma. Semin Diagn Pathol. 31(1):39-47, 2014
                                    8. Cote GM et al: Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin North Am. 27(5):1007-19, 2013
                                    9. Grohar PJ et al: Prospects and challenges for the development of new therapies for Ewing sarcoma. Pharmacol Ther. 137(2):216-24, 2013
                                    10. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                    11. Ross KA et al: The biology of ewing sarcoma. ISRN Oncol. 2013:759725, 2013
                                    12. 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
                                    13. Eary JF et al: Imaging in sarcoma. J Nucl Med. 52(12):1903-13, 2011
                                    14. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                    15. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                    16. 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
                                    17. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                    18. 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
                                    19. Riggi N et al: The Biology of Ewing sarcoma. Cancer Lett. 254(1):1-10, 2007
                                    20. Bernstein M et al: Ewing's sarcoma family of tumors: current management. Oncologist. 11(5):503-19, 2006
                                    21. 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
                                    22. 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

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Ewing sarcoma (ES)
                                            • Definitions

                                              • 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

                                              • 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. Huang T et al: Effectiveness of 18F-FDG PET/CT in the diagnosis, staging and recurrence monitoring of Ewing sarcoma family of tumors: A meta-analysis of 23 studies. Medicine (Baltimore). 97(48):e13457, 2018
                                                                      2. Ruggiero A et al: Diagnostic accuracy of 18F-FDG PET/CT in the staging and assessment of response to chemotherapy in children with Ewing sarcoma. J Pediatr Hematol Oncol. 40(4):277-84, 2018
                                                                      3. Palmerini E et al: The role of FDG PET/CT in patients treated with neoadjuvant chemotherapy for localized bone sarcomas. Eur J Nucl Med Mol Imaging. 44(2):215-23, 2017
                                                                      4. Hwang JP et al: Prognostic value of SUVmax measured by pretreatment fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Ewing sarcoma. PLoS One. 11(4):e0153281, 2016
                                                                      5. Raciborska A et al: Response to chemotherapy estimates by FDG PET is an important prognostic factor in patients with Ewing sarcoma. Clin Transl Oncol. 18(2):189-95, 2016
                                                                      6. Gelfand MJ et al: [F-18.] Pediatr Radiol. 45(8):1182-8, 2015
                                                                      7. Choi EY et al: Ewing sarcoma. Semin Diagn Pathol. 31(1):39-47, 2014
                                                                      8. Cote GM et al: Update in treatment and targets in Ewing sarcoma. Hematol Oncol Clin North Am. 27(5):1007-19, 2013
                                                                      9. Grohar PJ et al: Prospects and challenges for the development of new therapies for Ewing sarcoma. Pharmacol Ther. 137(2):216-24, 2013
                                                                      10. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                                                      11. Ross KA et al: The biology of ewing sarcoma. ISRN Oncol. 2013:759725, 2013
                                                                      12. 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
                                                                      13. Eary JF et al: Imaging in sarcoma. J Nucl Med. 52(12):1903-13, 2011
                                                                      14. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                                                      15. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                                                      16. 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
                                                                      17. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                                                      18. 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
                                                                      19. Riggi N et al: The Biology of Ewing sarcoma. Cancer Lett. 254(1):1-10, 2007
                                                                      20. Bernstein M et al: Ewing's sarcoma family of tumors: current management. Oncologist. 11(5):503-19, 2006
                                                                      21. 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
                                                                      22. 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