Document Type: Case Report


1 Dept. of Oral and Maxillofacial Surgery, Dental School, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

2 Dept. of Oral and Maxillofacial Pathology, School of Dentistry, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

3 Postgraduate Student, Dept. of Oral and Maxillofacial Pathology, Dental School Shahid Beheshti University of Medical Sciences, Tehran, Iran.


Ewing's sarcoma is an uncommon malignancy primarily affecting bone tissue and usually occurs in adolescents and young adults. This paper reports a rare case of extra-skeletal Ewing’s sarcoma of the oral cavity soft tissue. In the clinical examination, a mass of 1.5×1.5 cm in diameter was observed in the right mandibular vestibule. Radiographic examination revealed no involvement of mandible. Microscopically, the tumor was composed of malignant small round cell tumor that exhibited immunoreactivity for CD99. The patient was subjected to enucleation surgery under local anesthesia, prognosis was excellent, and the patient remained symptom-free after 13 months.


1. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer. 1975; 36: 240–251. [PubMed[Google Scholar]

2. Sinha DK, Jha NK, Yadav SK, Yadav J, Sinha R. Ewing's Sarcoma of Mandible: A Very Rare Disease and Review of Indian Literature. Indian J Surg Oncol. 2014; 5: 81–84. [PMC free article] [PubMed[Google Scholar]

3. Ko E, Brouns ER, Korones DN, Pochal WF, Philipone EM, Zegarelli DJ, et al. Primary Ewing sarcoma of the anterior mandible localized to the midline. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013; 115: e46–e50. [PubMed[Google Scholar]

4. Cho SI, Park YH, Cho JH, Ryoo BY, Yang SH, Youn SM, et al. Extraskeletal Ewing's sarcoma of the head and neck presenting as blindness. Korean J Intern Med. 2007; 22: 133–137. [PMC free article] [PubMed[Google Scholar]

5. Berk R, Heller A, Heller D, Schwartz S, Klein EA. Ewing's sarcoma of the mandible: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1995; 79: 159–162. [PubMed[Google Scholar]

6. Mukherjee A, Ray JG, Bhattacharya S, Deb T. Ewing's sarcoma of mandible: A case report and review of Indian literature. Contemp Clin Dent. 2012; 3: 494–498. [PMC free article] [PubMed[Google Scholar]

7. Wood RE, Nortje CJ, Hesseling P, Grotepass F. Ewing's tumor of the jaw. Oral Surg Oral Med Oral Pathol. 1990; 69: 120–127. [PubMed[Google Scholar]

8. Wang CL, Yacobi R, Pharoah M, Thorner P. Ewing's sarcoma: metastatic tumor to the jaw. Oral Surg Oral Med Oral Pathol. 1991; 71: 597–602. [PubMed[Google Scholar]

9. Talesh KT, Motamedi MH, Jeihounian M. Ewing's sarcoma of the mandibular condyle: report of a case. J Oral Maxillofac Surg. 2003; 61: 1216–1219. [PubMed[Google Scholar]

10. Ng SH, Ko SF, Cheung YC, Wong HF, Jung SM. Extraskeletal Ewing's sarcoma of the parapharyngeal space. Br J Radiol. 2004; 77: 1046–1049. [PubMed[Google Scholar]

11. Sandhya K, Sangeetha K, Balan A, Girija K, Bose T. Extraskeletal Ewing’s Sarcoma of Floor of Mouth: A 1-year follow-up of the Rare Disease in a Rare Location. International Journal of Scientific Study. 2017; 4: 220–223. [Google Scholar]

12. Narula MK, Gupta N, Anand R, Kapoor S. Extraosseous Ewing's sarcoma / primitive neuroectodermal tumor of the sacral nerve plexus. Indian J Radiol Imaging. 2009; 19: 151–154. [PMC free article] [PubMed[Google Scholar]

13. Tarazona N, Navarro L, Cejalvo JM, Gambardella V, Pérez-Fidalgo JA, Sempere A, et al. Primary paraesophageal Ewing's sarcoma: an uncommon case report and literature review. Onco Targets Ther. 2015; 8: 1053–1059. [PMC free article] [PubMed[Google Scholar]

14. Berková A, Dundr P, Povýsil C, Melcákova S, Tvrdík D. A comparison of RT-PCR and FISH techniques in molecular diagnosis of Ewing's sarcoma in paraffin-embedded tissue. Cesk Patol. 2008; 44: 67–70. [PubMed[Google Scholar]

15. Machado I, Noguera R, Pellin A, Lopez-Guerrero JA, Piqueras M, Navarro S, et al. Molecular diagnosis of Ewing sarcoma family of tumors: a comparative analysis of 560cases with FISH and RT-PCR. Diagn Mol Pathol. 2009; 18: 189–199. [PubMed[Google Scholar]