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Mandible-Maxilla Ewing Sarcoma
Axel Ruprecht, DDS, MScD, FRCD(C)
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Ewing sarcoma (ES)
            • Synonyms

              • ES/primitive neuroectodermal tumor (ES/PNET)
              • ES family of tumors (ESFT)
            • Definitions

              • Group of cancers including
                • Classic ES
                • PNET or primitive neuroepithelioma
                • Malignant small round cell tumor

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Leroy R et al: Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers. Int J Radiat Oncol Biol Phys. 95(1):267-78, 2016
                                      2. Qureshi SS et al: Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children. Int J Oral Maxillofac Surg. 45(8):938-44, 2016
                                      3. Grevener K et al: Management and Outcome of Ewing Sarcoma of the Head and Neck. Pediatr Blood Cancer. ePub, 2015
                                      4. Negru ME et al: Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report. Mol Clin Oncol. 3(4):807-810, 2015
                                      5. Ahmed AA et al: Therapy resistance mechanisms in Ewing's sarcoma family tumors. Cancer Chemother Pharmacol. 73(4):657-63, 2014
                                      6. Li M et al: Primary Ewing's sarcoma of the ethmoid sinus with intracranial and orbital extension: case report and literature review. Am J Otolaryngol. 34(5):563-8, 2013
                                      7. Yamaoka M et al: Multidisciplinary therapy including proton beam radiotherapy for a Ewing sarcoma family tumor of maxillary sinus in a 4-year-old girl. Head Neck. 35(12):E386-90, 2013
                                      8. Rombi B et al: Proton radiotherapy for pediatric Ewing's sarcoma: initial clinical outcomes. Int J Radiat Oncol Biol Phys. 82(3):1142-8, 2012
                                      9. Tanboon J et al: Primary intracranial Ewing sarcoma with an unusually aggressive course: a case report and review of the literature. Neuropathology. 32(3):293-300, 2012
                                      10. Womer RB et al: Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol. 30(33):4148-54, 2012
                                      11. Yeshvanth SK et al: Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Ther. 8(1):142-4, 2012
                                      12. Davido N et al: Case of Ewing's sarcoma misdiagnosed as a periapical lesion of maxillary incisor. J Endod. 37(2):259-64, 2011
                                      13. Gradoni P et al: Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children. Auris Nasus Larynx. 38(4):480-6, 2011
                                      14. Hayes SM et al: Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma. J Laryngol Otol. 125(8):861-4, 2011
                                      15. Brazão-Silva MT et al: Ewing's sarcoma of the mandible in a young child. Braz Dent J. 21(1):74-9, 2010
                                      16. Gradoni P et al: The role of surgery in children with head and neck rhabdomyosarcoma and Ewing's sarcoma. Surg Oncol. 19(4):e103-9, 2010
                                      17. Holsinger FC et al: Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. Ear Nose Throat J. 89(11):534-40, 2010
                                      18. Whaley JT et al: Ewing tumors of the head and neck. Am J Clin Oncol. 33(4):321-6, 2010
                                      19. Casiraghi O et al: [Undifferentiated malignant round cell tumors of the sinonasal tract and nasopharynx.] Ann Pathol. 29(4):296-312, 2009
                                      20. Gray ST et al: Efficacy of Proton Beam Therapy in the Treatment of Ewing's Sarcoma of the Paranasal Sinuses and Anterior Skull Base. Skull Base. 19(6):409-16, 2009
                                      21. Gupta S et al: Ewing sarcoma of the maxilla: a rare presentation. Quintessence Int. 40(2):135-40, 2009
                                      22. Erişir F et al: Ewing/Primitive neuroectodermal tumour family sarcoma arising from a branchial cyst. J Otolaryngol Head Neck Surg. 37(3):E96-9, 2008
                                      23. Kawabata M et al: Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus. Rhinology. 46(1):75-8, 2008
                                      24. Menezes AH: Craniovertebral junction neoplasms in the pediatric population. Childs Nerv Syst. 24(10):1173-86, 2008
                                      25. O'Sullivan PJ et al: Radiological features of synovial cell sarcoma. Br J Radiol. 81(964):346-56, 2008
                                      26. Solomon LW et al: Ewing sarcoma of the mandibular condyle: multidisciplinary management optimizes outcome. Head Neck. 30(3):405-10, 2008
                                      27. Thariat J et al: Very Late Local Relapse of Ewing's Sarcoma of the Head and Neck treated with Aggressive Multimodal Therapy. Sarcoma. 2008:854141, 2008
                                      28. Weinreb I et al: Primary extraskeletal Ewing family tumor with complex epithelial differentiation: a unique case arising in the lateral neck presenting with Horner syndrome. Am J Surg Pathol. 32(11):1742-8, 2008
                                      29. Agir H et al: Extra-skeletal Ewing's sarcoma of the submandibular gland. J Plast Reconstr Aesthet Surg. 60(12):1345-8, 2007
                                      30. Paulino AC et al: An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma. Pediatr Blood Cancer. 48(4):423-9, 2007
                                      31. Gaba RC et al: Metastatic Ewing sarcoma masquerading as olfactory neuroblastoma. Eur Arch Otorhinolaryngol. 263(10):960-2, 2006
                                      32. Iezzoni JC et al: "Undifferentiated" small round cell tumors of the sinonasal tract: differential diagnosis update. Am J Clin Pathol. 124 Suppl:S110-21, 2005
                                      33. Nakayama E et al: The clinical and diagnostic imaging findings of osteosarcoma of the jaw. Dentomaxillofac Radiol. 34(3):182-8, 2005
                                      34. Howarth KL et al: A sinonasal primary Ewing's sarcoma. Int J Pediatr Otorhinolaryngol. 68(2):221-4, 2004
                                      35. Windfuhr JP: Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. Ann Otol Rhinol Laryngol. 113(7):533-43, 2004
                                      36. Cope JU et al: Ewing sarcoma and sinonasal neuroectodermal tumors as second malignant tumors after retinoblastoma and other neoplasms. Med Pediatr Oncol. 36(2):290-4, 2001
                                      37. Allam A et al: Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases. Sarcoma. 3(1):11-5, 1999
                                      38. Fiorillo A et al: Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy. Cancer Lett. 103(2):177-82, 1996
                                      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

                                                • Abbreviations

                                                  • Ewing sarcoma (ES)
                                                • Synonyms

                                                  • ES/primitive neuroectodermal tumor (ES/PNET)
                                                  • ES family of tumors (ESFT)
                                                • Definitions

                                                  • Group of cancers including
                                                    • Classic ES
                                                    • PNET or primitive neuroepithelioma
                                                    • Malignant small round cell tumor

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Leroy R et al: Proton Therapy in Children: A Systematic Review of Clinical Effectiveness in 15 Pediatric Cancers. Int J Radiat Oncol Biol Phys. 95(1):267-78, 2016
                                                                          2. Qureshi SS et al: Local therapy in non-metastatic primary Ewing sarcoma of the mandible and maxilla in children. Int J Oral Maxillofac Surg. 45(8):938-44, 2016
                                                                          3. Grevener K et al: Management and Outcome of Ewing Sarcoma of the Head and Neck. Pediatr Blood Cancer. ePub, 2015
                                                                          4. Negru ME et al: Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report. Mol Clin Oncol. 3(4):807-810, 2015
                                                                          5. Ahmed AA et al: Therapy resistance mechanisms in Ewing's sarcoma family tumors. Cancer Chemother Pharmacol. 73(4):657-63, 2014
                                                                          6. Li M et al: Primary Ewing's sarcoma of the ethmoid sinus with intracranial and orbital extension: case report and literature review. Am J Otolaryngol. 34(5):563-8, 2013
                                                                          7. Yamaoka M et al: Multidisciplinary therapy including proton beam radiotherapy for a Ewing sarcoma family tumor of maxillary sinus in a 4-year-old girl. Head Neck. 35(12):E386-90, 2013
                                                                          8. Rombi B et al: Proton radiotherapy for pediatric Ewing's sarcoma: initial clinical outcomes. Int J Radiat Oncol Biol Phys. 82(3):1142-8, 2012
                                                                          9. Tanboon J et al: Primary intracranial Ewing sarcoma with an unusually aggressive course: a case report and review of the literature. Neuropathology. 32(3):293-300, 2012
                                                                          10. Womer RB et al: Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group. J Clin Oncol. 30(33):4148-54, 2012
                                                                          11. Yeshvanth SK et al: Rare case of extraskeletal Ewings sarcoma of the sinonasal tract. J Cancer Res Ther. 8(1):142-4, 2012
                                                                          12. Davido N et al: Case of Ewing's sarcoma misdiagnosed as a periapical lesion of maxillary incisor. J Endod. 37(2):259-64, 2011
                                                                          13. Gradoni P et al: Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children. Auris Nasus Larynx. 38(4):480-6, 2011
                                                                          14. Hayes SM et al: Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma. J Laryngol Otol. 125(8):861-4, 2011
                                                                          15. Brazão-Silva MT et al: Ewing's sarcoma of the mandible in a young child. Braz Dent J. 21(1):74-9, 2010
                                                                          16. Gradoni P et al: The role of surgery in children with head and neck rhabdomyosarcoma and Ewing's sarcoma. Surg Oncol. 19(4):e103-9, 2010
                                                                          17. Holsinger FC et al: Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. Ear Nose Throat J. 89(11):534-40, 2010
                                                                          18. Whaley JT et al: Ewing tumors of the head and neck. Am J Clin Oncol. 33(4):321-6, 2010
                                                                          19. Casiraghi O et al: [Undifferentiated malignant round cell tumors of the sinonasal tract and nasopharynx.] Ann Pathol. 29(4):296-312, 2009
                                                                          20. Gray ST et al: Efficacy of Proton Beam Therapy in the Treatment of Ewing's Sarcoma of the Paranasal Sinuses and Anterior Skull Base. Skull Base. 19(6):409-16, 2009
                                                                          21. Gupta S et al: Ewing sarcoma of the maxilla: a rare presentation. Quintessence Int. 40(2):135-40, 2009
                                                                          22. Erişir F et al: Ewing/Primitive neuroectodermal tumour family sarcoma arising from a branchial cyst. J Otolaryngol Head Neck Surg. 37(3):E96-9, 2008
                                                                          23. Kawabata M et al: Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus. Rhinology. 46(1):75-8, 2008
                                                                          24. Menezes AH: Craniovertebral junction neoplasms in the pediatric population. Childs Nerv Syst. 24(10):1173-86, 2008
                                                                          25. O'Sullivan PJ et al: Radiological features of synovial cell sarcoma. Br J Radiol. 81(964):346-56, 2008
                                                                          26. Solomon LW et al: Ewing sarcoma of the mandibular condyle: multidisciplinary management optimizes outcome. Head Neck. 30(3):405-10, 2008
                                                                          27. Thariat J et al: Very Late Local Relapse of Ewing's Sarcoma of the Head and Neck treated with Aggressive Multimodal Therapy. Sarcoma. 2008:854141, 2008
                                                                          28. Weinreb I et al: Primary extraskeletal Ewing family tumor with complex epithelial differentiation: a unique case arising in the lateral neck presenting with Horner syndrome. Am J Surg Pathol. 32(11):1742-8, 2008
                                                                          29. Agir H et al: Extra-skeletal Ewing's sarcoma of the submandibular gland. J Plast Reconstr Aesthet Surg. 60(12):1345-8, 2007
                                                                          30. Paulino AC et al: An analysis of primary site control and late effects according to local control modality in non-metastatic Ewing sarcoma. Pediatr Blood Cancer. 48(4):423-9, 2007
                                                                          31. Gaba RC et al: Metastatic Ewing sarcoma masquerading as olfactory neuroblastoma. Eur Arch Otorhinolaryngol. 263(10):960-2, 2006
                                                                          32. Iezzoni JC et al: "Undifferentiated" small round cell tumors of the sinonasal tract: differential diagnosis update. Am J Clin Pathol. 124 Suppl:S110-21, 2005
                                                                          33. Nakayama E et al: The clinical and diagnostic imaging findings of osteosarcoma of the jaw. Dentomaxillofac Radiol. 34(3):182-8, 2005
                                                                          34. Howarth KL et al: A sinonasal primary Ewing's sarcoma. Int J Pediatr Otorhinolaryngol. 68(2):221-4, 2004
                                                                          35. Windfuhr JP: Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. Ann Otol Rhinol Laryngol. 113(7):533-43, 2004
                                                                          36. Cope JU et al: Ewing sarcoma and sinonasal neuroectodermal tumors as second malignant tumors after retinoblastoma and other neoplasms. Med Pediatr Oncol. 36(2):290-4, 2001
                                                                          37. Allam A et al: Ewing's Sarcoma of the Head and Neck: A Retrospective Analysis of 24 Cases. Sarcoma. 3(1):11-5, 1999
                                                                          38. Fiorillo A et al: Primary Ewing's sarcoma of the maxilla, a rare and curable localization: report of two new cases, successfully treated by radiotherapy and systemic chemotherapy. Cancer Lett. 103(2):177-82, 1996