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Extraskeletal Ewing Sarcoma
Stephanie A. Bernard, MD; Catherine C. Roberts, MD
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KEY FACTS

  • Imaging

    • Pathology

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Extraskeletal Ewing sarcoma (EES)
          • Small round cell sarcoma (SRCS)
        • Synonyms

          • No longer recommended: peripheral neuroectodermal (PNET), peripheral neuroepithelioma, peripheral neuroblastoma, or Askin tumor
        • WHO Classification of Undifferentiated Small Round Cell Sarcomas

          • Ewing sarcoma
          • CIC-rearranged sarcoma
          • Sarcoma with BCOR alterations
          • Round cell sarcoma with EWSR1-non-ETS fusions: contains remainder of formerly Ewing-like undifferentiated sarcomas

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Angiographic Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Brady EJ et al: Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 50(3):521-9, 2021
                                      2. Sirisena UDN et al: Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 50(7):1291-301, 2021
                                      3. Brčić I et al: Undifferentiated round cell sarcomas with CIC-DUX4 gene fusion: expanding the clinical spectrum. Pathology. 52(2):236-42, 2020
                                      4. Antonescu CR et al: Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases. Am J Surg Pathol. 41(7):941-9, 2017
                                      5. Huh J et al: Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: a 17-year experience at a single institution. Korean J Radiol. 16(4):783-90, 2015
                                      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. Javery O et al: A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 197(6):W1015-22, 2011
                                      8. Bahk WJ et al: Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings. Skeletal Radiol. 39(6):595-600, 2010
                                      9. Weiss SW et al: Ewing's sarcoma/PNET tumor family and related lesions. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 963-79, 2008
                                      10. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 371-4, 2006
                                      11. Carvajal R et al: Ewing's sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am. 19(3):501-25, vi-vii, 2005
                                      12. Christie DR et al: Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol. 41(1):22-8, 1997
                                      13. Allam K et al: MR of primary extraosseous Ewing sarcoma. AJNR Am J Neuroradiol. 15(2):305-7, 1994
                                      14. Winer-Muram HT et al: Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings. AJR Am J Roentgenol. 161(2):265-8, 1993
                                      15. O'Keeffe F et al: Radiological features of extraskeletal Ewing sarcoma. Br J Radiol. 63(750):456-60, 1990
                                      16. Rose JS et al: Extraskeletal Ewing sarcoma with computed tomography correlation. Skeletal Radiol. 9(4):234-7, 1983
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Extraskeletal Ewing sarcoma (EES)
                                              • Small round cell sarcoma (SRCS)
                                            • Synonyms

                                              • No longer recommended: peripheral neuroectodermal (PNET), peripheral neuroepithelioma, peripheral neuroblastoma, or Askin tumor
                                            • WHO Classification of Undifferentiated Small Round Cell Sarcomas

                                              • Ewing sarcoma
                                              • CIC-rearranged sarcoma
                                              • Sarcoma with BCOR alterations
                                              • Round cell sarcoma with EWSR1-non-ETS fusions: contains remainder of formerly Ewing-like undifferentiated sarcomas

                                            IMAGING

                                            • General Features

                                              • Radiographic Findings

                                                • CT Findings

                                                  • MR Findings

                                                    • Ultrasonographic Findings

                                                      • Angiographic Findings

                                                        • Nuclear Medicine Findings

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Brady EJ et al: Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 50(3):521-9, 2021
                                                                          2. Sirisena UDN et al: Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 50(7):1291-301, 2021
                                                                          3. Brčić I et al: Undifferentiated round cell sarcomas with CIC-DUX4 gene fusion: expanding the clinical spectrum. Pathology. 52(2):236-42, 2020
                                                                          4. Antonescu CR et al: Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases. Am J Surg Pathol. 41(7):941-9, 2017
                                                                          5. Huh J et al: Imaging features of primary tumors and metastatic patterns of the extraskeletal Ewing sarcoma family of tumors in adults: a 17-year experience at a single institution. Korean J Radiol. 16(4):783-90, 2015
                                                                          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. Javery O et al: A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 197(6):W1015-22, 2011
                                                                          8. Bahk WJ et al: Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings. Skeletal Radiol. 39(6):595-600, 2010
                                                                          9. Weiss SW et al: Ewing's sarcoma/PNET tumor family and related lesions. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 963-79, 2008
                                                                          10. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 371-4, 2006
                                                                          11. Carvajal R et al: Ewing's sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am. 19(3):501-25, vi-vii, 2005
                                                                          12. Christie DR et al: Diagnostic difficulties in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas Radiol. 41(1):22-8, 1997
                                                                          13. Allam K et al: MR of primary extraosseous Ewing sarcoma. AJNR Am J Neuroradiol. 15(2):305-7, 1994
                                                                          14. Winer-Muram HT et al: Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings. AJR Am J Roentgenol. 161(2):265-8, 1993
                                                                          15. O'Keeffe F et al: Radiological features of extraskeletal Ewing sarcoma. Br J Radiol. 63(750):456-60, 1990
                                                                          16. Rose JS et al: Extraskeletal Ewing sarcoma with computed tomography correlation. Skeletal Radiol. 9(4):234-7, 1983