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Ewing Sarcoma
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Ewing sarcoma family of tumors (ESFT)
            • Round cell sarcoma of bone
          • Definitions

            • Aggressive childhood cancer which includes Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumor (PNET)

          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: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                            2. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                            3. Indelicato DJ et al: Spinal and paraspinal Ewing tumors. Int J Radiat Oncol Biol Phys. 76(5):1463-71, 2010
                                            4. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                            5. Klimo P Jr et al: Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 4(3):222-9, 2009
                                            6. Mar WA et al: Update on imaging and treatment of Ewing sarcoma family tumors: what the radiologist needs to know. J Comput Assist Tomogr. 32(1):108-18, 2008
                                            7. Bacci G et al: Long-term outcome for patients with non-metastatic Ewing's sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. Eur J Cancer. 40(1):73-83, 2004
                                            8. Caksen H et al: A case of metastatic spinal Ewing's sarcoma misdiagnosed as brucellosis and transverse myelitis. Neurol Sci. 24(6):414-6, 2004
                                            9. Bacci G et al: Therapy and survival after recurrence of Ewing's tumors: the Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. Ann Oncol. 14(11):1654-9, 2003
                                            10. Burchill SA: Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. J Clin Pathol. 56(2):96-102, 2003
                                            11. Fuchs B et al: Complications in long-term survivors of Ewing sarcoma. Cancer. 98(12):2687-92, 2003
                                            12. Fuchs B et al: Ewing's sarcoma and the development of secondary malignancies. Clin Orthop. (415):82-9, 2003
                                            13. Goktepe AS et al: Paraplegia: an unusual presentation of Ewing's sarcoma. Spinal Cord. 40(7):367-9, 2002
                                            14. 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
                                            15. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and ewing sarcoma. Top Magn Reson Imaging. 13(4):231-9, 2002
                                            16. Paulussen M et al: Ewing tumour: incidence, prognosis and treatment options. Paediatr Drugs. 3(12):899-913, 2001
                                            17. Devoe K et al: Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol. 17(3):216-24, 2000
                                            18. Llombart-Bosch A et al: Histology, immunohistochemistry, and electron microscopy of small round cell tumors of bone. Semin Diagn Pathol. 13(3):153-70, 1996
                                            19. Downing JR et al: Detection of the (11;22)(q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction. Am J Pathol. 143(5):1294-300, 1993
                                            20. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                            21. Boyko OB et al: MR imaging of osteogenic and Ewing's sarcoma. AJR Am J Roentgenol. 148(2):317-22, 1987
                                            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

                                                    • Synonyms

                                                      • Ewing sarcoma family of tumors (ESFT)
                                                      • Round cell sarcoma of bone
                                                    • Definitions

                                                      • Aggressive childhood cancer which includes Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumor (PNET)

                                                    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: From the radiologic pathology archives: ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                                                                      2. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                                                                      3. Indelicato DJ et al: Spinal and paraspinal Ewing tumors. Int J Radiat Oncol Biol Phys. 76(5):1463-71, 2010
                                                                                      4. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                                                                      5. Klimo P Jr et al: Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 4(3):222-9, 2009
                                                                                      6. Mar WA et al: Update on imaging and treatment of Ewing sarcoma family tumors: what the radiologist needs to know. J Comput Assist Tomogr. 32(1):108-18, 2008
                                                                                      7. Bacci G et al: Long-term outcome for patients with non-metastatic Ewing's sarcoma treated with adjuvant and neoadjuvant chemotherapies. 402 patients treated at Rizzoli between 1972 and 1992. Eur J Cancer. 40(1):73-83, 2004
                                                                                      8. Caksen H et al: A case of metastatic spinal Ewing's sarcoma misdiagnosed as brucellosis and transverse myelitis. Neurol Sci. 24(6):414-6, 2004
                                                                                      9. Bacci G et al: Therapy and survival after recurrence of Ewing's tumors: the Rizzoli experience in 195 patients treated with adjuvant and neoadjuvant chemotherapy from 1979 to 1997. Ann Oncol. 14(11):1654-9, 2003
                                                                                      10. Burchill SA: Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. J Clin Pathol. 56(2):96-102, 2003
                                                                                      11. Fuchs B et al: Complications in long-term survivors of Ewing sarcoma. Cancer. 98(12):2687-92, 2003
                                                                                      12. Fuchs B et al: Ewing's sarcoma and the development of secondary malignancies. Clin Orthop. (415):82-9, 2003
                                                                                      13. Goktepe AS et al: Paraplegia: an unusual presentation of Ewing's sarcoma. Spinal Cord. 40(7):367-9, 2002
                                                                                      14. 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
                                                                                      15. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and ewing sarcoma. Top Magn Reson Imaging. 13(4):231-9, 2002
                                                                                      16. Paulussen M et al: Ewing tumour: incidence, prognosis and treatment options. Paediatr Drugs. 3(12):899-913, 2001
                                                                                      17. Devoe K et al: Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol. 17(3):216-24, 2000
                                                                                      18. Llombart-Bosch A et al: Histology, immunohistochemistry, and electron microscopy of small round cell tumors of bone. Semin Diagn Pathol. 13(3):153-70, 1996
                                                                                      19. Downing JR et al: Detection of the (11;22)(q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction. Am J Pathol. 143(5):1294-300, 1993
                                                                                      20. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                                                                      21. Boyko OB et al: MR imaging of osteogenic and Ewing's sarcoma. AJR Am J Roentgenol. 148(2):317-22, 1987