Massive Pediatric Mandibular Ewing’s Sarcoma Presenting with Life-Threatening Airway Obstruction: A Case Report
Abstract
Ewing’s sarcoma is a rare and aggressive malignant small round-cell tumor that typically arises in the long bones and pelvis, with only 1–2% of cases involving the maxillofacial skeleton. Mandibular presentation is exceptionally uncommon and presents diagnostic and therapeutic challenges. This study reports a clinical case and includes a focused literature review. A 9-year-old girl presented with a large mandibular mass associated with airway compromise and recurrent intraoral bleeding. Clinical, radiological, and histopathological examinations were performed. The patient underwent urgent tracheostomy, near-total mandibulectomy with a lip-split incision and 2 cm margins, followed by immediate titanium plate reconstruction. Postoperative care included adjuvant multiagent chemotherapy. A literature review of cases reported between 1988 and 2025 was conducted for comparison. Surgery achieved complete tumor resection with immediate stabilization of the airway and mandibular continuity. Postoperative histopathology confirmed the diagnosis of Ewing’s sarcoma with clear margins. The patient recovered with stable airway function and initiated adjuvant chemotherapy. Review of published cases demonstrated variability in presentation, management, and survival outcomes. A summary of comparative mandibular cases is presented in Table 1. Pediatric mandibular Ewing’s sarcoma is rare but may present with airway emergencies. Early recognition, multidisciplinary planning, and prompt surgical intervention with airway protection are critical for favorable outcomes. Keywords: Airway management; Ewing sarcoma; Mandibular neoplasms; Maxillofacial surgery; Pediatric oncology; Reconstructive surgical procedures.
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2. Neville BW, Damm DD, Allen CM, Chi AC. Oral and Maxillofacial Pathology. 4th ed. St. Louis: Elsevier; 2016. p. 667–9.
3. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Dunst J, et al. Ewing’s sarcoma family of tumors: current management. Oncologist. 2006;11(5):503–19.
4. Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, et al. Survival in Ewing sarcoma: results of the EICESS-92 and Euro-E.W.I.N.G. 99 trials. Eur J Cancer. 2021;147:132–44.
5. Ahuja US, Puri N, Gupta D, Kumar S, Chaudhary S. Ewing’s sarcoma of mandible: a rare case report and review. Int J Clin Pediatr Dent. 2019;12(5):470–4.
6. Lopes SL, Almeida SM, Costa AL, Zanardi VA, Cendes F. Imaging findings of Ewing’s sarcoma in the mandible. J Oral Sci. 2007;49(2):167–71.
7. Gorospe L, Fernández-Gil MA, García-Raya P, Royo A, López-Barea F, García-Miguel P. Ewing’s sarcoma of the mandible: radiologic features with emphasis on magnetic resonance appearance. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;91(6):728–34.
8. Schultze-Mosgau S, Thorwarth M, Wehrhan F, Holter W, Stachel KD, Grabenbauer G. Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction. J Craniofac Surg. 2005;16(6):1140–6.
9. Gaspar N, Hawkins DS, Dirksen U, Lewis IJ, Ferrari S, Le Deley MC, et al. Ewing sarcoma: current management and future approaches. Nat Rev Clin Oncol. 2021;18(11):639–56.
10. Reddy GS, Reddy SV, Reddy DR, Mounika T, Devi RS. Ewing’s sarcoma of mandible: a rare case report and review. J Oral Maxillofac Surg. 2022;80(1):142–8.
11. Bellut N, Lutz CM, Lesnik M, Hähnel S, Probst FA. Mandibular Ewing sarcoma: a rare case report with review of the literature. J Craniomaxillofac Surg. 2024;52(5):450–4.
12. Bacci G, Ferrari S, Bertoni F, Ruggieri P, Picci P, Longhi A, et al. Prognostic factors in nonmetastatic Ewing’s sarcoma of bone: analysis of 579 patients. Cancer. 2000;89(4):799–807.
13. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma. N Engl J Med. 2003;348(8):694–701.
14. Miser JS, Goldsby RE, Chen Z, Krailo MD, Tarbell NJ, Link MP, et al. Localized Ewing sarcoma: alternating VDC/IE. J Clin Oncol. 2004;22(17):3423–30.
15. Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H, et al. Ewing sarcoma. Nat Rev Dis Primers. 2018;4:5.
16. Rao BHS, Hazarika M, Dhupar V. Ewing’s sarcoma of the mandible. J Maxillofac Oral Surg. 2011;10(1):64–8.
17. Casaroto AN, Toledo GL, Filho LC, Ricardo de Souza N, et al. Ewing’s sarcoma family tumors in the jaws: case report and literature review. In Vivo. 2017;31(3):481–6.
18. Suzuki T, Nakayama M, Yoshizaki T, Harabuchi Y. Primary Ewing’s sarcoma of the sinonasal tract: case report and review. Clin Exp Otorhinolaryngol. 2017;10(1):106–10.
19. Nakai Y, Kubo T, Inoue N, Miyazaki M, Sakamoto K, Okamoto H, et al. Treatment of mandibular Ewing’s sarcoma in a child. J Stomatol Oral Maxillofac Surg. 2023;124(1):101326.
20. Bernstein PE, Briggs R. Ewing’s sarcoma of the mandible. Arch Otolaryngol. 1979;105(9):538–41.
21. Van den Bergh JPA, van der Waal I. Ewing’s sarcoma of the mandible: report of a case. J Oral Maxillofac Surg. 1988;46(9):798–800.
22. Wang CC, Huang SC, Chen YK, Chen CH. Ewing’s sarcoma of the mandible: report of a case. J Oral Maxillofac Surg. 1991;49(5):532–6.
23. da Fonseca MA, Abrams RB. Ewing’s sarcoma of the mandible in a young patient: case report. Pediatr Dent. 1992;14(6):402–4.
24. Bessède JP, Monsaint B, Huth J, de Lumley L, Sauvage JP. Ewing’s sarcoma of the mandible: a case report. Br J Oral Maxillofac Surg. 1993;31(1):49–51.
25. Berk R, Heller M, Coral I, Buchbinder D. Ewing’s sarcoma of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995;80(5):561–6.
26. Vaccani JP, Forte V, de Jong AL, Taylor G. Ewing’s sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol. 1999;48(3):209–16.
27. Fonseca AS, Mezzalira R, Crespo AN, Bortoleto Junior AE, Paschoal JR. Ewing’s sarcoma of the head and neck: case report. Sao Paulo Med J. 2000;118(6):198–200.
28. Talesh KT, Motamedi MHK, Navi F. Ewing’s sarcoma of the mandibular condyle: report of a case. J Oral Maxillofac Surg. 2003;61(10):1216–9.
29. Lopes SL, Almeida SM, Costa AL, Zanardi VA, Cendes F. Imaging findings of Ewing’s sarcoma in the mandible. J Oral Sci. 2007;49(2):167–71.
30. Gosau M, Baumhoer D, Ihrler S, Kleinheinz J, Driemel O. Ewing sarcoma of the mandible mimicking an odontogenic abscess. Head Face Med. 2008;4:24.
31. Saleh M, Mahajan K, Singh K, et al. The oral surgeon in the front-line of the diagnosis of Ewing’s sarcoma of the mandible: a case report. J Oral Maxillofac Pathol. 2018;22(4):520–4.
32. Sinha PB, Prasad K, Mukherjee A, Das A, Das S. Primary Ewing’s sarcoma of body of mandible: multimodal treatment. Ann Case Rep. 2020;5:1–4.
33. Ram R, Prasad KR, Raju KV, Kumar R. Pediatric mandibular Ewing sarcoma with airway compromise: case report. J Pediatr Surg. 2023;In press.
34. Bellut N, Lutz CM, Lesnik M, Hähnel S, Probst FA. Ewing’s sarcoma of mandible: case report with literature review. Int J Clin Pediatr Dent. 2024;17(2):187–90.
2. Neville BW, Damm DD, Allen CM, Chi AC. Oral and Maxillofacial Pathology. 4th ed. St. Louis: Elsevier; 2016. p. 667–9.
3. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Dunst J, et al. Ewing’s sarcoma family of tumors: current management. Oncologist. 2006;11(5):503–19.
4. Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, et al. Survival in Ewing sarcoma: results of the EICESS-92 and Euro-E.W.I.N.G. 99 trials. Eur J Cancer. 2021;147:132–44.
5. Ahuja US, Puri N, Gupta D, Kumar S, Chaudhary S. Ewing’s sarcoma of mandible: a rare case report and review. Int J Clin Pediatr Dent. 2019;12(5):470–4.
6. Lopes SL, Almeida SM, Costa AL, Zanardi VA, Cendes F. Imaging findings of Ewing’s sarcoma in the mandible. J Oral Sci. 2007;49(2):167–71.
7. Gorospe L, Fernández-Gil MA, García-Raya P, Royo A, López-Barea F, García-Miguel P. Ewing’s sarcoma of the mandible: radiologic features with emphasis on magnetic resonance appearance. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001;91(6):728–34.
8. Schultze-Mosgau S, Thorwarth M, Wehrhan F, Holter W, Stachel KD, Grabenbauer G. Ewing sarcoma of the mandible in a child: interdisciplinary treatment concepts and surgical reconstruction. J Craniofac Surg. 2005;16(6):1140–6.
9. Gaspar N, Hawkins DS, Dirksen U, Lewis IJ, Ferrari S, Le Deley MC, et al. Ewing sarcoma: current management and future approaches. Nat Rev Clin Oncol. 2021;18(11):639–56.
10. Reddy GS, Reddy SV, Reddy DR, Mounika T, Devi RS. Ewing’s sarcoma of mandible: a rare case report and review. J Oral Maxillofac Surg. 2022;80(1):142–8.
11. Bellut N, Lutz CM, Lesnik M, Hähnel S, Probst FA. Mandibular Ewing sarcoma: a rare case report with review of the literature. J Craniomaxillofac Surg. 2024;52(5):450–4.
12. Bacci G, Ferrari S, Bertoni F, Ruggieri P, Picci P, Longhi A, et al. Prognostic factors in nonmetastatic Ewing’s sarcoma of bone: analysis of 579 patients. Cancer. 2000;89(4):799–807.
13. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma. N Engl J Med. 2003;348(8):694–701.
14. Miser JS, Goldsby RE, Chen Z, Krailo MD, Tarbell NJ, Link MP, et al. Localized Ewing sarcoma: alternating VDC/IE. J Clin Oncol. 2004;22(17):3423–30.
15. Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H, et al. Ewing sarcoma. Nat Rev Dis Primers. 2018;4:5.
16. Rao BHS, Hazarika M, Dhupar V. Ewing’s sarcoma of the mandible. J Maxillofac Oral Surg. 2011;10(1):64–8.
17. Casaroto AN, Toledo GL, Filho LC, Ricardo de Souza N, et al. Ewing’s sarcoma family tumors in the jaws: case report and literature review. In Vivo. 2017;31(3):481–6.
18. Suzuki T, Nakayama M, Yoshizaki T, Harabuchi Y. Primary Ewing’s sarcoma of the sinonasal tract: case report and review. Clin Exp Otorhinolaryngol. 2017;10(1):106–10.
19. Nakai Y, Kubo T, Inoue N, Miyazaki M, Sakamoto K, Okamoto H, et al. Treatment of mandibular Ewing’s sarcoma in a child. J Stomatol Oral Maxillofac Surg. 2023;124(1):101326.
20. Bernstein PE, Briggs R. Ewing’s sarcoma of the mandible. Arch Otolaryngol. 1979;105(9):538–41.
21. Van den Bergh JPA, van der Waal I. Ewing’s sarcoma of the mandible: report of a case. J Oral Maxillofac Surg. 1988;46(9):798–800.
22. Wang CC, Huang SC, Chen YK, Chen CH. Ewing’s sarcoma of the mandible: report of a case. J Oral Maxillofac Surg. 1991;49(5):532–6.
23. da Fonseca MA, Abrams RB. Ewing’s sarcoma of the mandible in a young patient: case report. Pediatr Dent. 1992;14(6):402–4.
24. Bessède JP, Monsaint B, Huth J, de Lumley L, Sauvage JP. Ewing’s sarcoma of the mandible: a case report. Br J Oral Maxillofac Surg. 1993;31(1):49–51.
25. Berk R, Heller M, Coral I, Buchbinder D. Ewing’s sarcoma of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995;80(5):561–6.
26. Vaccani JP, Forte V, de Jong AL, Taylor G. Ewing’s sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol. 1999;48(3):209–16.
27. Fonseca AS, Mezzalira R, Crespo AN, Bortoleto Junior AE, Paschoal JR. Ewing’s sarcoma of the head and neck: case report. Sao Paulo Med J. 2000;118(6):198–200.
28. Talesh KT, Motamedi MHK, Navi F. Ewing’s sarcoma of the mandibular condyle: report of a case. J Oral Maxillofac Surg. 2003;61(10):1216–9.
29. Lopes SL, Almeida SM, Costa AL, Zanardi VA, Cendes F. Imaging findings of Ewing’s sarcoma in the mandible. J Oral Sci. 2007;49(2):167–71.
30. Gosau M, Baumhoer D, Ihrler S, Kleinheinz J, Driemel O. Ewing sarcoma of the mandible mimicking an odontogenic abscess. Head Face Med. 2008;4:24.
31. Saleh M, Mahajan K, Singh K, et al. The oral surgeon in the front-line of the diagnosis of Ewing’s sarcoma of the mandible: a case report. J Oral Maxillofac Pathol. 2018;22(4):520–4.
32. Sinha PB, Prasad K, Mukherjee A, Das A, Das S. Primary Ewing’s sarcoma of body of mandible: multimodal treatment. Ann Case Rep. 2020;5:1–4.
33. Ram R, Prasad KR, Raju KV, Kumar R. Pediatric mandibular Ewing sarcoma with airway compromise: case report. J Pediatr Surg. 2023;In press.
34. Bellut N, Lutz CM, Lesnik M, Hähnel S, Probst FA. Ewing’s sarcoma of mandible: case report with literature review. Int J Clin Pediatr Dent. 2024;17(2):187–90.
| Files | ||
| Issue | Vol 12, No 4 (Autumn 2025) | |
| Section | Case Report(s) | |
| Keywords | ||
| Airway management; Ewing sarcoma; Mandibular neoplasms; Maxillofacial surgery; Pediatric oncology; Reconstructive surgical procedures. | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Jaza Mahmood B. Massive Pediatric Mandibular Ewing’s Sarcoma Presenting with Life-Threatening Airway Obstruction: A Case Report. J Craniomaxillofac Res. 2026;12(4):282-287.
