Iranian Journal of Otorhinolaryngology

Iranian Journal of Otorhinolaryngology

Primary Extraosseous Ewing Sarcoma of the Maxillary Sinus in an Adult-A Rare Case Report

Document Type : Case Report

Authors
Ein-Wan Chin 1
Azreen-Zaira Abu Bakar 1
Shahrul Hitam 1
Ngan Kah Wai 2
Maizaton-Atmadini Abdullah 3
1 Department of otorhinolaryngology,Ampang Hospital, Selangor, Malaysia.
2 Department of Pathology, Serdang Hospital, Serdang, Selangor, Malaysia.
3 Department of Pathology, Faculty of Medicine and Health Sciences, University Putra Malaysia (UPM, Serdang, Selangor)
10.22038/ijorl.2019.35555.2173
Abstract
Introduction:
Ewing sarcoma (ES), which is described as diffuse endothelioma of the bone, is divided into osseous and extraosseous Ewing sarcoma (EES) mostly affecting children and adolescents. It is a rare, aggressive, and poorly differentiated small blue round cell tumor that seldom affects the head and neck regions.
 
Case Report:
Herein, we reported a 46-year-old man presenting with right nasal block, epistaxis,  and epiphora from the right eye for one month. The nasal endoscopy revealed a friable mass  arising from the anterior half of the right nasal cavity. Histological findings were suggestive of  Ewing sarcoma. A contrast-enhanced computed tomography (CT) scan of the paranasal sinuses showed a soft tissue mass in the right anterior nasal cavity with mucosal thickening in the right maxillary sinus, without any bony erosion or distant metastasis. The patient underwent endoscopic medial maxillectomy with modified Denker’s procedure, followed by a 6-cycle course of chemotherapy. He was clinically well after chemotherapy; however, the recent bone scans were suggestive of bone involvement with the tumor.
 
Conclusion:
TheEES of paranasal sinus in the head and neck regions is extremely rare and requires exceptional attention due to their adjacent vital structures. The ES diagnosis-related dilemma  arises from the numerous differential diagnoses of small round blue cell tumors. In this regard, accurate diagnosis is important, since ES requires a multi-modality approach. Furthermore, early diagnosis and aggressive intervention are crucial to obtain good prognosis and function.
Keywords
Ewing
Epistaxis
Maxillary Sinus
Nasal Obstruction
Nasal Cavity
Sarcoma
Subjects
  1. Ewing J. Diffuse endothelioma of bone. Proc N Y Pathol Soc.1921;21:17-24.
  2. Angervakk L, Enzinger FM (1975) Extraskeletal neoplasm resembling Ewing sarcoma. Cancer 36:240–51.
  3. Siegal GP, Oliver WR, Reinus WR, 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.
  4. Hafezi S, Seethala RR, Stelow EB,  Mills SE, Leong IT, Elaine MacDuff, et al. Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone.Head and Neck Pathol(2011)5:8-16.
  5. Allam A, El-Husseiny G, Khafaga Y, Alaa Kandil, Alan Gray, Adnan Ezzat et al. Ewing’s sarcoma of the head and neck: a retrospective analysis of 24 cases. Sarcoma 1999; 3:11–15.
  6. Ushigome S, Machinami R, Sorensen PH. Ewing sarcoma/ primitive neuroectodermal tumor (PNET). In: Fletcher CD, Unni KK, Mertens F, editors. World Health Organization classification of tumours. Pathology and genetics of bone and soft tissue tumours Lyon. France: IARC Press; 2002. p. 298–300.
  7. Maria EN, Andrea PS, David R, Francesca P, Marco G, Laura F, et al. Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report.Mol Clin Oncol. 2015 Jul; 3(4): 807-10. Published online 2015 Apr24. PMCID: PMC4487037.
  8. Dutta M, Ghatak S, Biswas G, Sen A. Primary soft tissue Ewing's sarcoma of the maxillary sinus in elderly patients: presentation, management and prognosis. Singapore Medical Journal [01 Jun 2014, 55(6):e96-100]. PMID: 24442190. PMCID: PMC4294066.
  9. Mark Aferzon, W. Edward W, James RP. Ewing’s sarcoma of the ethmoid sinus. Otolaryngol Head Neck Surg 2003;128:897-901.
  10. Varshney S, Bist SS, Gupta N,  Bhatia R.  Primary extraskeletal Ewing’s sarcoma of the maxilla with intraorbital extension. Indian J Otolaryngol Head Neck Surg. 2007 Sep; 59(3):273–6.
  11. Caner O, Taner A, Altan Y, Feramuz D, Meltem N, Oznur D. Primary Ewing’s sarcoma of the paranasal sinus with intracranial and intraorbital extension. European Journal of Radiology Extra 55 (2005) 47–50.
  12. Firas N, Moustafa A, Ousama A, Emaad B. Ewing’s Sarcoma of the maxillary sinus. Egyptian Journal of Ear, Nose, Throat and Allied Sciences (2015) 16; 177-180.
  13. 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–E117.
  14. Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, et al. Ewing’s sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol. 1997;15(2):574–82.
  15. Le Deley MC, Paulussen M, Lewis I, Brennan B, Ranft A, Whelan J, et al. Cyclophosphamide compared with ifosfamide in consolidation treatment of standard-risk Ewing sarcoma: Results of the randomized noninferiority Euro-EWING99-R1 trial. J Clin Oncol 2014; 32: 2440–8.
  16. Kathryn I. Pontius, MD. Bruce A. Sebek, MD. Extraskeletal Ewing's Sarcoma Arising in the Nasal Fossa: Light- and Electron-microscopic Observations. American Journal of Clinical Pathology, Volume 75, Issue 3, 1 March 1981, Pages 410–15.
  17. Davide L, Davide M, Luca O, Remo A, Maria L, Fabio F, et al. Primary Ewing's sarcoma of the sinonasal tract in adults: A challenging disease. Head & Neck Journals of the sciences and specialties of the head and neck, volume 39, issue3, March 2017, pages E45-E50.
  18. Suzuki T, Yasumatsu R, Nakashima T, Arita S, Yamamoto H,  Nakagawaa T. Primary Ewing's Sarcoma of the Sinonasal Tract: A Case Report. Case Rep Oncol. 2017 Jan-Apr;10(1):91–7.
  19. Kissane JM, Askin FB, Foulkes M, Stratton LB. Shirley SF. Ewing’s sarcoma of bone: Clinicopathologic aspects of 303 cases from the Intergroup Ewing’s Sarcoma Study. Hum Pathol 1983; 14:773- 9.
  20. Lane S, Ironside JW. Extraskeletal Ewing’s sarcoma of the nasal fossa. J Laryngol Otol 1990;104: 570–3.
Iranian Journal of Otorhinolaryngology
Volume 31, Issue 6 - Serial Number 107
November and December 2019
Pages 391-397