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Ewing Sarcoma
Kevin R. Moore, MD; Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

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

              • Aggressive childhood cancer that 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. Berger GK et al: Outcomes in different age groups with primary Ewing sarcoma of the spine: a systematic review of the literature. J Neurosurg Spine. 1-10, 2019
                                              2. Hesla AC et al: Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years. J Bone Oncol. 14:100216, 2019
                                              3. Charest-Morin R et al: Ewing sarcoma of the spine: prognostic variables for survival and local control in surgically treated patients. Spine (Phila Pa 1976). 43(9):622-9, 2018
                                              4. Scantland JT et al: Primary spinal intradural extraosseous Ewing sarcoma in a pediatric patient: case report and review of the literature. Pediatr Neurosurg. 53(4):222-8, 2018
                                              5. Murphey MD et al: From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                              6. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                              7. Indelicato DJ et al: Spinal and paraspinal Ewing tumors. Int J Radiat Oncol Biol Phys. 76(5):1463-71, 2010
                                              8. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                              9. Klimo P Jr et al: Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 4(3):222-9, 2009
                                              10. 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
                                              11. 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
                                              12. 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
                                              13. 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
                                              14. Burchill SA: Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. J Clin Pathol. 56(2):96-102, 2003
                                              15. Fuchs B et al: Complications in long-term survivors of Ewing sarcoma. Cancer. 98(12):2687-92, 2003
                                              16. Fuchs B et al: Ewing's sarcoma and the development of secondary malignancies. Clin Orthop. (415):82-9, 2003
                                              17. Goktepe AS et al: Paraplegia: an unusual presentation of Ewing's sarcoma. Spinal Cord. 40(7):367-9, 2002
                                              18. 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
                                              19. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and Ewing sarcoma. Top Magn Reson Imaging. 13(4):231-9, 2002
                                              20. Paulussen M et al: Ewing tumour: incidence, prognosis and treatment options. Paediatr Drugs. 3(12):899-913, 2001
                                              21. Devoe K et al: Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol. 17(3):216-24, 2000
                                              22. 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
                                              23. 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
                                              24. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                              25. 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

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Synonyms

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

                                                          • Aggressive childhood cancer that 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. Berger GK et al: Outcomes in different age groups with primary Ewing sarcoma of the spine: a systematic review of the literature. J Neurosurg Spine. 1-10, 2019
                                                                                          2. Hesla AC et al: Ewing sarcoma of the mobile spine; predictive factors for survival, neurological function and local control. A Scandinavian sarcoma group study with a mean follow-up of 12 years. J Bone Oncol. 14:100216, 2019
                                                                                          3. Charest-Morin R et al: Ewing sarcoma of the spine: prognostic variables for survival and local control in surgically treated patients. Spine (Phila Pa 1976). 43(9):622-9, 2018
                                                                                          4. Scantland JT et al: Primary spinal intradural extraosseous Ewing sarcoma in a pediatric patient: case report and review of the literature. Pediatr Neurosurg. 53(4):222-8, 2018
                                                                                          5. Murphey MD et al: From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 33(3):803-31, 2013
                                                                                          6. Balamuth NJ et al: Ewing's sarcoma. Lancet Oncol. 11(2):184-92, 2010
                                                                                          7. Indelicato DJ et al: Spinal and paraspinal Ewing tumors. Int J Radiat Oncol Biol Phys. 76(5):1463-71, 2010
                                                                                          8. Maheshwari AV et al: Ewing sarcoma family of tumors. J Am Acad Orthop Surg. 18(2):94-107, 2010
                                                                                          9. Klimo P Jr et al: Primary pediatric intraspinal sarcomas. Report of 3 cases. J Neurosurg Pediatr. 4(3):222-9, 2009
                                                                                          10. 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
                                                                                          11. 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
                                                                                          12. 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
                                                                                          13. 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
                                                                                          14. Burchill SA: Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. J Clin Pathol. 56(2):96-102, 2003
                                                                                          15. Fuchs B et al: Complications in long-term survivors of Ewing sarcoma. Cancer. 98(12):2687-92, 2003
                                                                                          16. Fuchs B et al: Ewing's sarcoma and the development of secondary malignancies. Clin Orthop. (415):82-9, 2003
                                                                                          17. Goktepe AS et al: Paraplegia: an unusual presentation of Ewing's sarcoma. Spinal Cord. 40(7):367-9, 2002
                                                                                          18. 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
                                                                                          19. Hoffer FA: Primary skeletal neoplasms: osteosarcoma and Ewing sarcoma. Top Magn Reson Imaging. 13(4):231-9, 2002
                                                                                          20. Paulussen M et al: Ewing tumour: incidence, prognosis and treatment options. Paediatr Drugs. 3(12):899-913, 2001
                                                                                          21. Devoe K et al: Immunohistochemistry of small round-cell tumors. Semin Diagn Pathol. 17(3):216-24, 2000
                                                                                          22. 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
                                                                                          23. 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
                                                                                          24. Eggli KD et al: Ewing's sarcoma. Radiol Clin North Am. 31(2):325-37, 1993
                                                                                          25. Boyko OB et al: MR imaging of osteogenic and Ewing's sarcoma. AJR Am J Roentgenol. 148(2):317-22, 1987