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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

Introduction:

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

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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

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