Extraskeletal Ewing’s Sarcoma of Neck in a Child- A Case Report

Document Type: Case Report


Department of Otorhinolaryngology, Dr. Shankarrao Chavan Government Medical College, Nanded, Maharashtra, India.


Ewing's sarcoma (ES) is an extremely rare bone malignancy observed in otorhinolaryngeal practice. In otorhinolaryngology, it sometimes involves the facial bones and cervical vertebrae. In children, ES is the second most common primary malignancy of bone after osteosarcoma. Extraskeletal Ewing’s sarcoma (EES) is an extremely rare malignancy of mesenchymal cell origin. The EES is observed in the trunk and lower extremities. The neck is an extremely rare primary site for EES.
Case Report:
A 3-year-old female child referred with a rapidly enlarging right-sided neck swelling since 2 months ago. Physical examination and preoperative investigations suggested the diagnosis of a carotid body tumor and histopathological findings were similar. However, the results of immunohistochemistry revealed a confirmatory diagnosis of ES.
Hereby, we presented a case of EES of the neck in a child, which was completely misdiagnosed preoperatively due to its rare incidence. The incidence of EES in the head and neck region can be successfully managed with radical excision and radiotherapy.


Main Subjects

1. Prindull G, Willert HG, Notter G. Local therapy of rhabdomyosarcoma, osteosarcoma and Ewing's Sarcoma of children and adolescents. Eur J Pediatr 1985;144(2):120-4.

2. Alvarez A, Schut L, Bruce D. Localized primary intracranial Ewing’s sarcoma of the orbital roof. Case report. J Neurosurg1979; 50:811- 3.

3. O’Connell JE, Calder C, Raafat F, Proops D. Ewing’s sarcoma of the retropharynx. The Journal of Laryngology and Otology 1994; 108:363- 6.

4. Hadfield MG, Luo VY, Williams RL, Ward JD, Russo CP. Ewing’s sarcoma of the skull in an infant. Pediatr Neurosurg1996; 25:100- 4.

5. Seigal GP, Oliver WR, Reinus WR, et al. Primary Ewing’s sarcoma involving the bones of the head and neck, Cancer1987; 60:2829- 40.

6. Tefft M, Vawter GF, Mitus A. Paravertebral "round cell" tumors in children. Radiology 1969. 92:1501–1509.

7. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing's sarcoma. Cancer 1975. 36:240–251.

8. Chao TK, Chang YL, Sheen TS. Extraskeletal Ewing's sarcoma of the scalp. J Laryngol Otol 2000. 114:73–75.

9. Rud NP, Reiman HM, Pritchard DJ, Frassica FJ, Smithson WA.Extraosseous Ewing’s sarcoma: A study of 42 cases. Cancer 1989; 64(7):1548-53.

10. Raney RB, Asmar L, Newton WA Jr, et al. Ewing’s sarcoma soft tissues in childhood: A report from the intergroup rhabdomyosarcoma study 1972 to 1991. J Clin Onc 1997; 15(2):574-82.