Skip to main content

Advertisement

SpringerLink
Log in

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

  • Original Paper
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

The Ewing’s family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7–70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1–168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. 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.

  2. Cotterill SJ, Ahrens S, Paulussen M, Jürgens HF, Voûte PA, Gadner H, Craft AW. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing’s Sarcoma study group. J Clin Oncol. 2000;18(17):3108–14.

    CAS  PubMed  Google Scholar 

  3. Siegal GP, Oliver WR, Reinus WR, Gilula LA, Foulkes MA, Kissane JM, Askin FB. Primary Ewing’s sarcoma involving the bones of the head and neck. Cancer. 1987;60(11):2829–40.

    Article  CAS  PubMed  Google Scholar 

  4. 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(3):209–16.

    Article  CAS  PubMed  Google Scholar 

  5. Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, Gehan EA, Webber B, Wharam M, Wiener ES, Anderson JR, Maurer HM. 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.

    CAS  PubMed  Google Scholar 

  6. Wenig BM, Dulguerov P, Kapadia SP, Prasad ML, Fanburg-smith JC Thompson LD. Neuroectodermal tumors. In: Barnes EL, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours. Pathology and genetics of head and neck tumours Lyon. France: IARC Press; 2005. p. 65–70.

  7. La TH, Meyers PA, Wexler LH, Alektiar KM, Healey JH, Laquaglia MP, Boland PJ, Wolden SL. Radiation therapy for Ewing’s sarcoma: results from Memorial Sloan-Kettering in the modern era. Int J Radiat Oncol Biol Phys. 2006;64(2):544–50.

    Article  PubMed  Google Scholar 

  8. Windfuhr JP. Primitive neuroectodermal tumor of the head and neck: incidence, diagnosis, and management. Ann Otol Rhinol Laryngol. 2004;113(7):533–43.

    PubMed  Google Scholar 

  9. Stelow EB, Bellizzi AM, Taneja K, Mills SE, Legallo RD, Kutok JL, Aster JC, French CA. NUT rearrangement in undifferentiated carcinomas of the upper aerodigestive tract. Am J Surg Pathol. 2008;32(6):828–34.

    Article  PubMed  Google Scholar 

  10. Wenig BM. Undifferentiated malignant neoplasms of the sinonasal tract. Arch Pathol Lab Med. 2009;133(5):699–712.

    PubMed  Google Scholar 

  11. Llombart-Bosch A, Machado I, Navarro S, Bertoni F, Bacchini P, Alberghini M, Karzeladze A, Savelov N, Petrov S, Alvarado-Cabrero I, Mihaila D, Terrier P, Lopez-Guerrero JA, Picci P. Histological heterogeneity of Ewing’s sarcoma/PNET: an immunohistochemical analysis of 415 genetically confirmed cases with clinical support. Virchows Arch. 2009;455(5):397–411.

    Article  CAS  PubMed  Google Scholar 

  12. Bahrami A, Gown AM, Baird GS, Hicks MJ, Folpe AL. Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall. Mod Pathol. 2008;21(7):795–806.

    Article  CAS  PubMed  Google Scholar 

  13. Infante-Cossio P, Gutierrez-Perez JL, Garcia-Perla A, Noguer-Mediavilla M, Gavilan-Carrasco F. Primary Ewing’s sarcoma of the maxilla and zygoma: report of a case. J Oral Maxillofac Surg. 2005;63(10):1539–42.

    Article  PubMed  Google Scholar 

  14. Argani P, Perez-Ordoñez B, Xiao H, Caruana SM, Huvos AG, Ladanyi M. Olfactory neuroblastoma is not related to the Ewing family of tumors: absence of EWS/FLI1 gene fusion and MIC2 expression. Am J Surg Pathol. 1998;22(4):391–8.

    Article  CAS  PubMed  Google Scholar 

  15. Whang-Peng J, Freter CE, Knutsen T, Nanfro JJ, Gazdar A. Translocation t(11;22) in esthesioneuroblastoma. Cancer Genet Cytogenet. 1987;29(1):155–7.

    Article  CAS  PubMed  Google Scholar 

  16. Sorensen PH, Wu JK, Berean KW, Lim JF, Donn W, Frierson HF, Reynolds CP, López-Terrada D, Triche TJ. Olfactory neuroblastoma is a peripheral primitive neuroectodermal tumor related to Ewing sarcoma. Proc Natl Acad Sci USA. 1996;93(3):1038–43.

    Article  CAS  PubMed  Google Scholar 

  17. Cordes B, Williams MD, Tirado Y, Bell D, Rosenthal DI, Al-Dhahri SF, Hanna EY, El-Naggar AK. Molecular and phenotypic analysis of poorly differentiated sinonasal neoplasms: an integrated approach for early diagnosis and classification. Hum Pathol. 2009;40(3):283–92.

    Article  CAS  PubMed  Google Scholar 

  18. Allam A, El-Husseiny G, Khafaga Y, Kandil A, Gray A, Ezzat A, Schultz H. Ewing’s Sarcoma of the head and neck: a retrospective analysis of 24 cases. Sarcoma. 1999;3(1):11–5.

    Article  CAS  PubMed  Google Scholar 

  19. Aferzon M, Wood WE, Powell JR. Ewing’s sarcoma of the ethmoid sinus. Otolaryngol Head Neck Surg. 2003;128(6):897–901.

    Article  PubMed  Google Scholar 

  20. Alobid I, Bernal-Sprekelsen M, Alos L, Benitez P, Traserra J, Mullol J. Peripheral primitive neuroectodermal tumour of the left maxillary sinus. Acta Otolaryngol. 2003;123(6):776–8.

    Article  PubMed  Google Scholar 

  21. Amin MN, Islam KM, Ahmed AN, Datta PG, Amin AS, Abdullah M. Ewing’s sarcoma of maxilla-a case report. Bangladesh Med Res Counc Bull. 1990;16(1):42–5.

    CAS  PubMed  Google Scholar 

  22. Böör A, Jurkovic I, Friedmann I, Plank L, Kocan P. Extraskeletal Ewing’s sarcoma of the nose. J Laryngol Otol. 2001;115(1):74–6.

    Article  PubMed  Google Scholar 

  23. Coskun BU, Cinar U, Savk H, Basak T, Dadas B. Isolated maxillary sinus Ewing’s sarcoma. Rhinology. 2005;43(3):225–8.

    PubMed  Google Scholar 

  24. Csokonai LV, Liktor B, Arató G, Helffrich F. Ewing’s sarcoma in the nasal cavity. Otolaryngol Head Neck Surg. 2001;125(6):665–7.

    Article  CAS  PubMed  Google Scholar 

  25. Fernandez CH, Lindberg RD, Sutow WW, Samuels ML. Localized Ewing’s sarcoma–treatment and results. Cancer. 1974;34(1):143–8.

    Article  CAS  PubMed  Google Scholar 

  26. Gray ST, Chen YL, Lin DT. Efficacy of proton beam therapy in the treatment of Ewing’s sarcoma of the paranasal sinuses and anterior skull base. Skull Base. 2009;19(6):409–16.

    Article  PubMed  Google Scholar 

  27. Gupta S, Gupta OP, Mehrotra S, Mehrotra D. Ewing sarcoma of the maxilla: a rare presentation. Quintessence Int. 2009;40(2):135–40.

    PubMed  Google Scholar 

  28. Harman M, Kiroglu F, Kosem M, Unal O. Primary Ewing’s sarcoma of the paranasal sinus with intracranial extension: imaging features. Dentomaxillofac Radiol. 2003;32(5):343–6.

    Article  CAS  PubMed  Google Scholar 

  29. Hossfeld DK, Seeber S, Siemers E, Schmidt CG, Scherer E. Early results of combined modality therapy of patients with Ewing’s sarcoma. Recent Results Cancer Res. 1982;80:124–7.

    CAS  PubMed  Google Scholar 

  30. Howard DJ, Daniels HA. Ewing’s sarcoma of the nose. Ear Nose Throat J. 1993;72(4):277–9.

    CAS  PubMed  Google Scholar 

  31. Howard DJ, Lund VJ. Primary Ewing’s sarcoma of the ethmoid bone. J Laryngol Otol. 1985;99(10):1019–23.

    Article  CAS  PubMed  Google Scholar 

  32. Howarth KL, Khodaei I, Karkanevatos A, Clarke RW. A sinonasal primary Ewing’s sarcoma. Int J Pediatr Otorhinolaryngol. 2004;68(2):221–4.

    Article  CAS  PubMed  Google Scholar 

  33. Kawabata M, Yoshifuku K, Sagara Y, Kurono Y. Ewing’s sarcoma/primitive neuroectodermal tumour occurring in the maxillary sinus. Rhinology. 2008;46(1):75–8.

    PubMed  Google Scholar 

  34. Lane S, Ironside JW. Extra-skeletal Ewing’s sarcoma of the nasal fossa. J Laryngol Otol. 1990;104(7):570–3.

    Article  CAS  PubMed  Google Scholar 

  35. Pontius KI, Sebek BA. Extraskeletal Ewing’s sarcoma arising in the nasal fossa. Light- and electron-microscopic observations. Am J Clin Pathol. 1981;75(3):410–5.

    Google Scholar 

  36. Strong LC, Herson J, Osborne BM, Sutow WW. Risk of radiation-related subsequent malignant tumors in survivors of Ewing’s sarcoma. J Natl Cancer Inst. 1979;62(6):1401–6.

    CAS  PubMed  Google Scholar 

  37. Thariat J, Italiano A, Peyrade F, Birtwisle-Peyrottes I, Gastaud L, Dassonville O, Thyss A. Very late local relapse of Ewing’s sarcoma of the head and neck treated with aggressive multimodal therapy. Sarcoma. 2008;2008:854141.

    Article  CAS  PubMed  Google Scholar 

  38. Velche-Haag B, Dehesdin D, Proust F, Marie JP, Andrieu-Guitrancourt J, Laquerriere A. Ewing’s sarcoma of the head and neck: a case report. Ann Otolaryngol Chir Cervicofac. 2002;119(6):363–8.

    CAS  PubMed  Google Scholar 

  39. Wexler LH, Kacker A, Piro JD, Haddad J Jr, Close LG. Combined modality treatment of Ewing’s sarcoma of the maxilla. Head Neck. 2003;25(2):168–72.

    Article  PubMed  Google Scholar 

  40. Whaley JT, Indelicato DJ, Morris CG, Hinerman RW, Amdur RJ, Mendenhall WM, Keole SR, Marcus RB Jr. Ewing Tumors of the Head and Neck. Am J Clin Oncol. 2009 Oct 16. [Epub ahead of print].

  41. Woodruff G, Thorner P, Skarf B. Primary Ewing’s sarcoma of the orbit presenting with visual loss. Br J Ophthalmol. 1988;72(10):786–92.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, University Health Network, Toronto, ON, Canada

    Sara Hafezi, Bayardo Perez-Ordoñez & Ilan Weinreb

  2. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Sara Hafezi, Bayardo Perez-Ordoñez & Ilan Weinreb

  3. Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Raja R. Seethala

  4. Department of Pathology, University of Virginia, Charlottesville, VA, USA

    Edward B. Stelow & Stacey E. Mills

  5. Departments of Pathology and Dentistry, Mount Sinai Hospital, Toronto, ON, Canada

    Iona T. Leong

  6. Department of Pathology, Western Infirmary, Glasgow, UK

    Elaine MacDuff

  7. Department of Pathology, Massachusetts General Hospital, Boston, MA, USA

    Jennifer L. Hunt

Authors
  1. Sara Hafezi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Raja R. Seethala
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Edward B. Stelow
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Stacey E. Mills
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Iona T. Leong
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Elaine MacDuff
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Jennifer L. Hunt
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Bayardo Perez-Ordoñez
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Ilan Weinreb
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ilan Weinreb.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hafezi, S., Seethala, R.R., Stelow, E.B. et al. Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone. Head and Neck Pathol 5, 8–16 (2011). https://doi.org/10.1007/s12105-010-0227-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-010-0227-x

Keywords

Search

Navigation