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Ewing Sarcoma
Christopher G. Anton, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Malignant primary bone tumor
          • Ewing sarcoma (EWS) family of tumors: Ewing, primitive neuroectodermal tumor (PNET), Askin tumor, extraosseous Ewing
        • Definitions

          • Aggressive small round blue cell tumor that typically arises in bone

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • 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

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Murphey MD et al: Staging and classification of primary musculoskeletal bone and soft tissue tumors based on the 2020 WHO update, from the AJR special series on cancer staging. AJR Am J Roentgenol. ePub, 2021
                                          2. Parlak Ş et al: Diffusion-weighted imaging for the differentiation of Ewing sarcoma from osteosarcoma. Skeletal Radiol. 50(10):2023-30, 2021
                                          3. Harrison DJ et al: PET with 18F-Fluorodeoxyglucose/computed tomography in the management of pediatric sarcoma. PET Clin. 15(3):333-47, 2020
                                          4. Saleh MM et al: Multiparametric MRI with diffusion-weighted imaging in predicting response to chemotherapy in cases of osteosarcoma and Ewing's sarcoma. Br J Radiol. 93(1115):20200257, 2020
                                          5. Tal AL et al: The utility of 18FDG PET/CT versus bone scan for identification of bone metastases in a pediatric sarcoma population and a review of the literature. J Pediatr Hematol Oncol. 43(2):52-8, 2020
                                          6. Veselis CA et al: Bone tumors occurring in the soft tissues: a review of the clinical, imaging, and histopathologic findings. Curr Probl Diagn Radiol. 50(3):419-29, 2020
                                          7. Marina N et al: Age, tumor characteristics, and treatment regimen as event predictors in Ewing: a children's oncology group report. Sarcoma. 2015:927123, 2015
                                          8. McCarville MB et al: Distinguishing osteomyelitis from Ewing sarcoma on radiography and MRI. AJR Am J Roentgenol. 205(3):640-50; quiz 651, 2015
                                          9. Orr WS et al: Analysis of prognostic factors in extraosseous Ewing sarcoma family of tumors: review of St. Jude Children's Research Hospital experience. Ann Surg Oncol. 19(12):3816-22, 2012
                                          10. Olmos D et al: Targeting the insulin-like growth factor 1 receptor in Ewing's sarcoma: reality and expectations. Sarcoma. 2011:402508, 2011
                                          11. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                          12. Mody RJ et al: FDG PET imaging of childhood sarcomas. Pediatr Blood Cancer. 54(2):222-7, 2010
                                          13. Charest M et al: FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases. Eur J Nucl Med Mol Imaging. 36(12):1944-51, 2009
                                          14. Peersman B et al: Ewing's sarcoma: imaging features. JBR-BTR. 90(5):368-76, 2007
                                          15. Hayashida Y et al: Monitoring therapeutic responses of primary bone tumors by diffusion-weighted image: Initial results. Eur Radiol. 16(12):2637-43, 2006
                                          16. Kumar R et al: Role of PET/PET-CT in the management of sarcomas. Expert Rev Anticancer Ther. 6(8):1241-50, 2006
                                          17. Schuetze SM et al: Use of positron emission tomography in localized extremity soft tissue sarcoma treated with neoadjuvant chemotherapy. Cancer. 103(2):339-48, 2005
                                          18. Kutluk MT et al: Treatment results and prognostic factors in Ewing sarcoma. Pediatr Hematol Oncol. 21(7):597-610, 2004
                                          19. Hawkins DS et al: Evaluation of chemotherapy response in pediatric bone sarcomas by [F-18]-fluorodeoxy-D-glucose positron emission tomography. Cancer. 94(12):3277-84, 2002
                                          20. PDQ Pediatric Treatment Editorial Board: Ewing Sarcoma Treatment (PDQ®): Health Professional Version, 2002
                                          21. Lang P et al: Musculoskeletal neoplasm: perineoplastic edema versus tumor on dynamic postcontrast MR images with spatial mapping of instantaneous enhancement rates. Radiology. 197(3):831-9, 1995
                                          22. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                          23. Reinus WR et al: Prognostic features of Ewing sarcoma on plain radiograph and computed tomography scan after initial treatment. A Pediatric Oncology Group study (8346). Cancer. 72(8):2503-10, 1993
                                          24. Rud NP et al: Extraosseous Ewing's sarcoma. A study of 42 cases. Cancer. 64(7):1548-53, 1989
                                          25. Reinus WR et al: Radiology of Ewing's sarcoma; intergroup Ewing's Sarcoma Study (IESS). RadioGraphics. 4:929-44, 1984
                                          26. Lombardi F et al: Ewing's sarcoma: an approach to radiological diagnosis. Tumori. 65(3):389-99, 1979
                                          27. Ewing Sarcoma Treatment (PDQ®)--Health Professional Version
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Malignant primary bone tumor
                                                  • Ewing sarcoma (EWS) family of tumors: Ewing, primitive neuroectodermal tumor (PNET), Askin tumor, extraosseous Ewing
                                                • Definitions

                                                  • Aggressive small round blue cell tumor that typically arises in bone

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • 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

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Murphey MD et al: Staging and classification of primary musculoskeletal bone and soft tissue tumors based on the 2020 WHO update, from the AJR special series on cancer staging. AJR Am J Roentgenol. ePub, 2021
                                                                                  2. Parlak Ş et al: Diffusion-weighted imaging for the differentiation of Ewing sarcoma from osteosarcoma. Skeletal Radiol. 50(10):2023-30, 2021
                                                                                  3. Harrison DJ et al: PET with 18F-Fluorodeoxyglucose/computed tomography in the management of pediatric sarcoma. PET Clin. 15(3):333-47, 2020
                                                                                  4. Saleh MM et al: Multiparametric MRI with diffusion-weighted imaging in predicting response to chemotherapy in cases of osteosarcoma and Ewing's sarcoma. Br J Radiol. 93(1115):20200257, 2020
                                                                                  5. Tal AL et al: The utility of 18FDG PET/CT versus bone scan for identification of bone metastases in a pediatric sarcoma population and a review of the literature. J Pediatr Hematol Oncol. 43(2):52-8, 2020
                                                                                  6. Veselis CA et al: Bone tumors occurring in the soft tissues: a review of the clinical, imaging, and histopathologic findings. Curr Probl Diagn Radiol. 50(3):419-29, 2020
                                                                                  7. Marina N et al: Age, tumor characteristics, and treatment regimen as event predictors in Ewing: a children's oncology group report. Sarcoma. 2015:927123, 2015
                                                                                  8. McCarville MB et al: Distinguishing osteomyelitis from Ewing sarcoma on radiography and MRI. AJR Am J Roentgenol. 205(3):640-50; quiz 651, 2015
                                                                                  9. Orr WS et al: Analysis of prognostic factors in extraosseous Ewing sarcoma family of tumors: review of St. Jude Children's Research Hospital experience. Ann Surg Oncol. 19(12):3816-22, 2012
                                                                                  10. Olmos D et al: Targeting the insulin-like growth factor 1 receptor in Ewing's sarcoma: reality and expectations. Sarcoma. 2011:402508, 2011
                                                                                  11. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                                                                  12. Mody RJ et al: FDG PET imaging of childhood sarcomas. Pediatr Blood Cancer. 54(2):222-7, 2010
                                                                                  13. Charest M et al: FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases. Eur J Nucl Med Mol Imaging. 36(12):1944-51, 2009
                                                                                  14. Peersman B et al: Ewing's sarcoma: imaging features. JBR-BTR. 90(5):368-76, 2007
                                                                                  15. Hayashida Y et al: Monitoring therapeutic responses of primary bone tumors by diffusion-weighted image: Initial results. Eur Radiol. 16(12):2637-43, 2006
                                                                                  16. Kumar R et al: Role of PET/PET-CT in the management of sarcomas. Expert Rev Anticancer Ther. 6(8):1241-50, 2006
                                                                                  17. Schuetze SM et al: Use of positron emission tomography in localized extremity soft tissue sarcoma treated with neoadjuvant chemotherapy. Cancer. 103(2):339-48, 2005
                                                                                  18. Kutluk MT et al: Treatment results and prognostic factors in Ewing sarcoma. Pediatr Hematol Oncol. 21(7):597-610, 2004
                                                                                  19. Hawkins DS et al: Evaluation of chemotherapy response in pediatric bone sarcomas by [F-18]-fluorodeoxy-D-glucose positron emission tomography. Cancer. 94(12):3277-84, 2002
                                                                                  20. PDQ Pediatric Treatment Editorial Board: Ewing Sarcoma Treatment (PDQ®): Health Professional Version, 2002
                                                                                  21. Lang P et al: Musculoskeletal neoplasm: perineoplastic edema versus tumor on dynamic postcontrast MR images with spatial mapping of instantaneous enhancement rates. Radiology. 197(3):831-9, 1995
                                                                                  22. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                                                                  23. Reinus WR et al: Prognostic features of Ewing sarcoma on plain radiograph and computed tomography scan after initial treatment. A Pediatric Oncology Group study (8346). Cancer. 72(8):2503-10, 1993
                                                                                  24. Rud NP et al: Extraosseous Ewing's sarcoma. A study of 42 cases. Cancer. 64(7):1548-53, 1989
                                                                                  25. Reinus WR et al: Radiology of Ewing's sarcoma; intergroup Ewing's Sarcoma Study (IESS). RadioGraphics. 4:929-44, 1984
                                                                                  26. Lombardi F et al: Ewing's sarcoma: an approach to radiological diagnosis. Tumori. 65(3):389-99, 1979
                                                                                  27. Ewing Sarcoma Treatment (PDQ®)--Health Professional Version