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Ewing Sarcoma
B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Ewing sarcoma (ES)
        • Synonyms

          • Peripheral neuroepithelioma, peripheral neuroblastoma, Askin tumor, primitive neuroectodermal tumor (PNET)
        • Definitions

          • Round cell sarcoma showing varying degrees of neuroectodermal differentiation (based on light microscopy, immunohistochemistry, or electron microscopy)
          • ES family of tumors: Cytogenetically closely related if not identical lesions on morphologic continuum
            • ES: Bone and rare soft tissue location
            • Primitive neuroectodermal tumor: Bone & soft tissue tumors with microscopic features indistinguishable from ES but with histologic rosette formation
            • Askin: Similar lesion, but involving chest wall

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  • Nuclear Medicine Findings

                    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. Brasme JF et al: Time to diagnosis of Ewing tumors in children and adolescents is not associated with metastasis or survival: a prospective multicenter study of 436 patients. J Clin Oncol. 32(18):1935-40, 2014
                                        2. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                        3. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                        4. Bestic JM et al: Use of FDG PET in staging, restaging, and assessment of therapy response in Ewing sarcoma. Radiographics. 29(5):1487-500, 2009
                                        5. Jawad MU et al: Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: an analysis of 1631 cases from the SEER database, 1973-2005. Cancer. 115(15):3526-36, 2009
                                        6. Kleis M et al: Diagnostic value of PET/CT for the staging and restaging of pediatric tumors. Eur J Nucl Med Mol Imaging. 36(1):23-36, 2009
                                        7. Lessnick SL et al: Small round cell sarcomas. Semin Oncol. 36(4):338-46, 2009
                                        8. Ordóñez JL et al: Advances in Ewing's sarcoma research: where are we now and what lies ahead? Cancer Res. 69(18):7140-50, 2009
                                        9. Reichardt W et al: Diffusion-weighted imaging as predictor of therapy response in an animal model of Ewing sarcoma. Invest Radiol. 44(5):298-303, 2009
                                        10. Suvà ML et al: Identification of cancer stem cells in Ewing's sarcoma. Cancer Res. 69(5):1776-81, 2009
                                        11. van den Berg H et al: Extra-osseous Ewing sarcoma. Pediatr Hematol Oncol. 26(4):175-85, 2009
                                        12. Wagner TD et al: Combination short-course preoperative irradiation, surgical resection, and reduced-field high-dose postoperative irradiation in the treatment of tumors involving the bone. Int J Radiat Oncol Biol Phys. 73(1):259-66, 2009
                                        13. Armbruster C et al: Ewing's sarcoma and peripheral primitive neuroectodermal tumor in adults: different features of a rare neoplasm. Onkologie. 31(4):179-84, 2008
                                        14. Esiashvili N et al: Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results data. J Pediatr Hematol Oncol. 30(6):425-30, 2008
                                        15. Indelicato DJ et al: Definitive radiotherapy for ewing tumors of extremities and pelvis: long-term disease control, limb function, and treatment toxicity. Int J Radiat Oncol Biol Phys. 72(3):871-7, 2008
                                        16. Leavey PJ et al: Ewing sarcoma: prognostic criteria, outcomes and future treatment. Expert Rev Anticancer Ther. 8(4):617-24, 2008
                                        17. Leavey PJ et al: Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatr Blood Cancer. 51(3):334-8, 2008
                                        18. 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
                                        19. Meyer JS et al: Imaging guidelines for children with Ewing sarcoma and osteosarcoma: a report from the Children's Oncology Group Bone Tumor Committee. Pediatr Blood Cancer. 51(2):163-70, 2008
                                        20. Navid F et al: Second cancers in patients with the Ewing sarcoma family of tumours. Eur J Cancer. 44(7):983-91, 2008
                                        21. Ushigome S et al: Ewing sarcoma/Primitive neuroectodermal tumour (PNET). In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumors of Soft Tissue and Bone. Lyon: IARC Press. 298-300, 2002
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Ewing sarcoma (ES)
                                              • Synonyms

                                                • Peripheral neuroepithelioma, peripheral neuroblastoma, Askin tumor, primitive neuroectodermal tumor (PNET)
                                              • Definitions

                                                • Round cell sarcoma showing varying degrees of neuroectodermal differentiation (based on light microscopy, immunohistochemistry, or electron microscopy)
                                                • ES family of tumors: Cytogenetically closely related if not identical lesions on morphologic continuum
                                                  • ES: Bone and rare soft tissue location
                                                  • Primitive neuroectodermal tumor: Bone & soft tissue tumors with microscopic features indistinguishable from ES but with histologic rosette formation
                                                  • Askin: Similar lesion, but involving chest wall

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        • Nuclear Medicine Findings

                                                          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. Brasme JF et al: Time to diagnosis of Ewing tumors in children and adolescents is not associated with metastasis or survival: a prospective multicenter study of 436 patients. J Clin Oncol. 32(18):1935-40, 2014
                                                                              2. Murphey MD et al: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                                                              3. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                                                              4. Bestic JM et al: Use of FDG PET in staging, restaging, and assessment of therapy response in Ewing sarcoma. Radiographics. 29(5):1487-500, 2009
                                                                              5. Jawad MU et al: Ewing sarcoma demonstrates racial disparities in incidence-related and sex-related differences in outcome: an analysis of 1631 cases from the SEER database, 1973-2005. Cancer. 115(15):3526-36, 2009
                                                                              6. Kleis M et al: Diagnostic value of PET/CT for the staging and restaging of pediatric tumors. Eur J Nucl Med Mol Imaging. 36(1):23-36, 2009
                                                                              7. Lessnick SL et al: Small round cell sarcomas. Semin Oncol. 36(4):338-46, 2009
                                                                              8. Ordóñez JL et al: Advances in Ewing's sarcoma research: where are we now and what lies ahead? Cancer Res. 69(18):7140-50, 2009
                                                                              9. Reichardt W et al: Diffusion-weighted imaging as predictor of therapy response in an animal model of Ewing sarcoma. Invest Radiol. 44(5):298-303, 2009
                                                                              10. Suvà ML et al: Identification of cancer stem cells in Ewing's sarcoma. Cancer Res. 69(5):1776-81, 2009
                                                                              11. van den Berg H et al: Extra-osseous Ewing sarcoma. Pediatr Hematol Oncol. 26(4):175-85, 2009
                                                                              12. Wagner TD et al: Combination short-course preoperative irradiation, surgical resection, and reduced-field high-dose postoperative irradiation in the treatment of tumors involving the bone. Int J Radiat Oncol Biol Phys. 73(1):259-66, 2009
                                                                              13. Armbruster C et al: Ewing's sarcoma and peripheral primitive neuroectodermal tumor in adults: different features of a rare neoplasm. Onkologie. 31(4):179-84, 2008
                                                                              14. Esiashvili N et al: Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results data. J Pediatr Hematol Oncol. 30(6):425-30, 2008
                                                                              15. Indelicato DJ et al: Definitive radiotherapy for ewing tumors of extremities and pelvis: long-term disease control, limb function, and treatment toxicity. Int J Radiat Oncol Biol Phys. 72(3):871-7, 2008
                                                                              16. Leavey PJ et al: Ewing sarcoma: prognostic criteria, outcomes and future treatment. Expert Rev Anticancer Ther. 8(4):617-24, 2008
                                                                              17. Leavey PJ et al: Prognostic factors for patients with Ewing sarcoma (EWS) at first recurrence following multi-modality therapy: A report from the Children's Oncology Group. Pediatr Blood Cancer. 51(3):334-8, 2008
                                                                              18. 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
                                                                              19. Meyer JS et al: Imaging guidelines for children with Ewing sarcoma and osteosarcoma: a report from the Children's Oncology Group Bone Tumor Committee. Pediatr Blood Cancer. 51(2):163-70, 2008
                                                                              20. Navid F et al: Second cancers in patients with the Ewing sarcoma family of tumours. Eur J Cancer. 44(7):983-91, 2008
                                                                              21. Ushigome S et al: Ewing sarcoma/Primitive neuroectodermal tumour (PNET). In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumors of Soft Tissue and Bone. Lyon: IARC Press. 298-300, 2002