Extraosseous Ewing’s Sarcoma of the Parapharyngeal Space -A Rare Entity - with Review of Literature

Document Type : Case Report


1 Department of Radiotherapy, Government Medical College and Hospital, Chandigarh, India.

2 Department of Pathology, Government Medical College and Hospital, Chandigarh, India.

3 Department of Otorhinolaryngology and Head and Neck Surgery, Government Medical College and Hospital, Chandigarh, India.


Extraosseous Ewing’s sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing’s sarcoma of the parapharyngeal space is even rarer. To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature.
Case Report:
We report a rare case of EES of the parapharyngeal space in an 8-year-old girl. She presented with complaints of earache, difficulty in breathing and swallowing and bleeding from the mouth. Investigations revealed a large parapharyngeal mass causing narrowing of the nasopharyngeal and oropharyngeal airway with skeletal and lung metastasis. Biopsy from the parapharyngeal mass was suggestive of malignant small round cell tumor. The patient was treated with chemotherapy and radiotherapy, but developed brain metastasis and succumbed to disease approximately 1 year after diagnosis. Herein, we describe the characteristic clinicopathological features and treatment with a comprehensive review of the literature.
EES in this unusual location behaves aggressively, with a high rate of recurrence and distant metastasis. Aggressive multimodal treatment comprising of multi-agent chemotherapy, surgical resection if feasible, and radiotherapy should be considered.


Main Subjects

1. Lessnick SL, Ladanyi M. Molecular pathogenesis of Ewing sarcoma: new therapeutic and transcriptional targets. Annu Rev Pathol 2012; 7:145–59.
2. Vaccani JP, Forte V, de Jong AL, Taylor G. Ewing’s sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol 1999;48:209–16.
3. Allam A, El-Husseiny G, Khafaga Y,  Kandil AGray AEzzat A, et al. Ewing’s sarcoma of the head and neck: a retrospective analysis of 24 cases. Sarcoma 1999; 3:11–5.
4. Abdel Rahman H, El-Baradie T, El-Baradie M, Bahaa S, Shalan M. Management head and neck Ewing’s sarcoma family of tumors: experience of the National Cancer Institute, Cairo University. J Egypt Natl Canc Inst 2010;22:41–7.
5. Biswas B, Thakar A, Mohanti BK, Vishnubhatla S, Bakhshi S. Prognostic factors in head and neck Ewing sarcoma family of tumors. Laryngoscope 2015; 125:E112–7.
6. Siegal GP, Oliver WR, ReinusWR, Gilula LA, Foulkes MA, Kissane JM, et al. Primary Ewing’s sarcoma involving the bones of the head and neck. Cancer 1987;60:2829–40.
7. Ng SH, Ko SF, Cheung YC, Wong HF, Jung SM. Extraskeletal Ewing's sarcoma of the parapharyngeal space. Br J Radiol 2004;77:1046–9.
8. Chaudhary N, Gupta D, Natesh V, Singh S. Extraskeletal Ewing's sarcoma of parapharyngeal space. Int J Pediatr Otorhinolaryngol Extra 2010; 5:159–61.
9. Ramos-Rivera G, Adler E, Ramesh KH, Schiff B, Suhrland M, Khader S. Extraskeletal Ewing sarcoma of the parapharyngeal space with a unique translocation, t(19;22) (q13.4;q12.2). Human Pathology: Case Reports 2016; 4:38–41.
10. Grevener KHaveman LMRanft AVan den Berg HJung SLadenstein R, et al. Management and outcome of the Ewing sarcoma of the head and neck. Pediatr Blood Cancer 2016; 63:604–10.