Skip to main content
×
Home

Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma

  • S M Hayes (a1), T N Jani (a2), S M Rahman (a2), S Jogai (a3), P G Harries (a1) and R J Salib (a1)...
Abstract
AbstractIntroduction:

Ewing's sarcoma is a rare, malignant tumour predominantly affecting young adolescent males. We describe a unique case of an isolated extra-skeletal metastasis from a skeletal Ewing's sarcoma primary, arising in the right sinonasal cavity of a young man who presented with severe epistaxis and periorbital cellulitis.

Results:

Histologically, the lesion comprised closely packed, slightly diffuse, atypical cells with round, hyperchromatic nuclei, scant cytoplasm and occasional mitotic figures, arranged in a sheet-like pattern. Immunohistochemical analysis showed positive staining only for cluster of differentiation 99 glycoprotein. Fluorescent in situ hybridisation identified the Ewing's sarcoma gene, confirming the diagnosis.

Management:

Complete surgical resection was achieved via a minimally invasive endoscopic transnasal approach; post-operative radiotherapy. Ten months post-operatively, there were no endoscopic or radiological signs of disease.

Conclusion:

Metastatic Ewing's sarcoma within the head and neck is incredibly rare and can pose significant diagnostic and therapeutic challenges. An awareness of different clinical presentations and distinct histopathological features is important to enable early diagnosis. This case illustrates one potential management strategy, and reinforces the evolving role of endoscopic transnasal approaches in managing sinonasal cavity and anterior skull base tumours.

Copyright
Corresponding author
Address for correspondence: Mr Stephen Hayes, c/o ENT Department, Southampton University Hospitals NHS Trust, Tremona Road, Southampton SO16 6YD, UK Fax: +44 (0)238 079 4868 E-mail: drhayes@doctors.net.uk
References
Hide All
1Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc 1921;21:1724
2Siegal GP, Olivier WR, Reinus WR. Primary Ewing's sarcoma involving the bones of the head and neck. Cancer 1987;60:2829–40
3Boor A, Jurkovic I, Friedmann I, Plank L, Kocan P. Extraskeletal Ewing's sarcoma of the nose. J Laryngol Otol 2001;115:74–6
4Tefft M, Vawter GF, Mitus A. Paravertebral ‘round cell’ tumours in children. Radiology 1969;92:1501–9
5Vaccanti 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
6Chao TK, Chang Y-L, Sheen TS. Extraskeletal Ewing's sarcoma of the scalp. J Laryngol Otol 2000;114:72–5
7Aferzon M, Wood WE, Powell JR. Ewing's sarcoma of the ethmoid sinus. Otolaryngol Head Neck Surg 2003;128:897901
8Hunsuck EE. Ewing's sarcoma of the maxilla. Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1968;25:923–8
9Howard DJ, Lund VJ. Primary Ewing's sarcoma of the ethmoid bone. J Laryngol Otol 1985;99:1019–23
10Beraud R, Fortin P. Ewing's sarcoma of the temporal localisation. Can Med Assoc J 1967;97:338–41
11Csokonai LV, Liktor B, Arato G, Helffrich F. Ewing's sarcoma in the nasal cavity. Otolaryngol Head Neck Surg 2001;125:665–7
12Batsakis JB, Mackay B, El-Naggar AK. Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report. Ann Otol Rhinol Laryngol 1996;105:838–43
13Grier HE. The Ewing family of tumours: Ewing's sarcoma and primitive neuroectodermal tumours. Pediatr Clin North Am 1997;15:2611–21
14Wenig BM, Prasad ML, Dulfuerov P, Fanburg-Smith JC, Kapadia SB, Thompson LDR. Neuroectodermal tumours. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. World Health Organization Classification of Tumours. Pathology & Genetics of Head & Neck Tumours. Lyon: IARC Press, 2005;65–6
15Lane S, Ironside JW. Extraskeletal Ewing's sarcoma of the nasal fossa. J Laryngol Otol 1990;104:570–3
16Howard DJ, Daniels HA. Ewing's sarcoma of the nose. Ear Nose Throat J 1993;72:277–9
17Kawabata M, Yoshifuku K, Sagara Y, Kurono Y. Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus. Rhinology 2008;46:75–8
18Yalcin S, Turoglu HT, Ozdamar S. Ewing's tumor of the mandible. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1993;76:362–7
19Dehner LP. Primitive neuroectodermal tumour and Ewing's sarcoma. Am J Surg Pathol 1993;17:113
20Coskun BU, Cinar U, Savk H, Basak T, Dadas B. Isolated maxillary sinus Ewing's sarcoma. Rhinology 2005;43:225–8
Recommend this journal

Email your librarian or administrator to recommend adding this journal to your organisation's collection.

The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
Please enter your name
Please enter a valid email address
Who would you like to send this to? *
×

Keywords:

Metrics

Full text views

Total number of HTML views: 1
Total number of PDF views: 6 *
Loading metrics...

Abstract views

Total abstract views: 91 *
Loading metrics...

* Views captured on Cambridge Core between September 2016 - 19th November 2017. This data will be updated every 24 hours.