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

Published online by Cambridge University Press:  18 May 2011

S M Hayes ,
T N Jani ,
S M Rahman ,
S Jogai ,
P G Harries  and
R J Salib
S M Hayes*
Affiliation:
Department of ENT, Southampton University Hospitals NHS Trust, UK
T N Jani
Affiliation:
Department of ENT, Salisbury NHS Foundation Trust, UK
S M Rahman
Affiliation:
Department of ENT, Salisbury NHS Foundation Trust, UK
S Jogai
Affiliation:
Department of Histopathology, Southampton University Hospitals NHS Trust, UK
P G Harries
Affiliation:
Department of ENT, Southampton University Hospitals NHS Trust, UK
R J Salib
Affiliation:
Department of ENT, Southampton University Hospitals NHS Trust, UK
*
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: [email protected]
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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.

Keywords

SarcomaEwing'sNeuroectodermal TumoursDiagnosisNeoplasm MetastasisPathologyRadiologyTherapeutics
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 125 , Issue 8 , August 2011 , pp. 861 - 864
Copyright
Copyright © JLO (1984) Limited 2011

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References

1Ewing, J. Diffuse endothelioma of bone. Proc N Y Pathol Soc 1921;21:17–24Google Scholar
2Siegal, GP, Olivier, WR, Reinus, WR. Primary Ewing's sarcoma involving the bones of the head and neck. Cancer 1987;60:2829–403.0.CO;2-S>CrossRefGoogle ScholarPubMed
3Boor, A, Jurkovic, I, Friedmann, I, Plank, L, Kocan, P. Extraskeletal Ewing's sarcoma of the nose. J Laryngol Otol 2001;115:74–6Google Scholar
4Tefft, M, Vawter, GF, Mitus, A. Paravertebral ‘round cell’ tumours in children. Radiology 1969;92:1501–9Google Scholar
5Vaccanti, JP, Forte, V, de Jong, AL, Taylor, G. Ewing's sarcoma of the head and neck in children. Int J Pediatr Otorhinolaryngol 1999;48:209–16CrossRefGoogle Scholar
6Chao, TK, Chang, Y-L, Sheen, TS. Extraskeletal Ewing's sarcoma of the scalp. J Laryngol Otol 2000;114:72–5Google Scholar
7Aferzon, M, Wood, WE, Powell, JR. Ewing's sarcoma of the ethmoid sinus. Otolaryngol Head Neck Surg 2003;128:897–901Google Scholar
8Hunsuck, EE. Ewing's sarcoma of the maxilla. Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1968;25:923–8CrossRefGoogle ScholarPubMed
9Howard, DJ, Lund, VJ. Primary Ewing's sarcoma of the ethmoid bone. J Laryngol Otol 1985;99:1019–23CrossRefGoogle ScholarPubMed
10Beraud, R, Fortin, P. Ewing's sarcoma of the temporal localisation. Can Med Assoc J 1967;97:338–41Google Scholar
11Csokonai, LV, Liktor, B, Arato, G, Helffrich, F. Ewing's sarcoma in the nasal cavity. Otolaryngol Head Neck Surg 2001;125:665–7CrossRefGoogle ScholarPubMed
12Batsakis, JB, Mackay, B, El-Naggar, AK. Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report. Ann Otol Rhinol Laryngol 1996;105:838–43CrossRefGoogle ScholarPubMed
13Grier, HE. The Ewing family of tumours: Ewing's sarcoma and primitive neuroectodermal tumours. Pediatr Clin North Am 1997;15:2611–21Google Scholar
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;65–6Google Scholar
15Lane, S, Ironside, JW. Extraskeletal Ewing's sarcoma of the nasal fossa. J Laryngol Otol 1990;104:570–3CrossRefGoogle ScholarPubMed
16Howard, DJ, Daniels, HA. Ewing's sarcoma of the nose. Ear Nose Throat J 1993;72:277–9Google Scholar
17Kawabata, M, Yoshifuku, K, Sagara, Y, Kurono, Y. Ewing's sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus. Rhinology 2008;46:75–8Google ScholarPubMed
18Yalcin, S, Turoglu, HT, Ozdamar, S. Ewing's tumor of the mandible. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1993;76:362–7Google Scholar
19Dehner, LP. Primitive neuroectodermal tumour and Ewing's sarcoma. Am J Surg Pathol 1993;17:1–13CrossRefGoogle ScholarPubMed
20Coskun, BU, Cinar, U, Savk, H, Basak, T, Dadas, B. Isolated maxillary sinus Ewing's sarcoma. Rhinology 2005;43:225–8Google Scholar