International Ophthalmology

, Volume 34, Issue 5, pp 1137–1140 | Cite as

Primary Ewing’s sarcoma of the orbit with intracranial extension abutting the temporal lobe: a rare case report

  • Syed Hassan Abbas Naqvi
  • Saad Hameed
  • Syed Hassan Shiraz Naqvi
  • Muhammad Anis Musani
Case Report


Ewing’s sarcoma is a small, blue, round-cell tumor of mesenchymal origin which typically presents itself during the first and the second decades of life. Typically, it is noticed in the long bones of the limbs, pelvis, or ribs. There have been few reports worldwide with none in Pakistan of this rare phenomenon. We report here the case of a 16-year-old female diagnosed with Ewing’s sarcoma with intracranial extension abutting the temporal lobe resulting in proptosis and partial loss of vision in the left eye. The purpose of this case is to discuss the clinicoradiological presentation, microscopic description, and management of the patient.


Ewing’s sarcoma Intracranial Diagnosis Management 



The authors like to thank the patient for providing consent to publish this case. We also appreciate the Radiology Department of Jinnah Postgraduate Medical Center and Agha Khan Hospital for providing the images necessary to complete this report. We also thank Muhammad Yasin Bandukda who assisted in editing this manuscript.

Conflict of interest

All authors declare that no competing interests exist.


  1. 1.
    Lou E, Sumrall AL, Cummings TJ, Korones DN, Weaver SA, Peters KB (2012) Disseminated intracranial Ewing’s sarcoma in an adult: a rare and difficult diagnosis. Case Rep Oncol 5(2):325–331PubMedCentralPubMedCrossRefGoogle Scholar
  2. 2.
    Arndt CA, Crist WM (1999) Common musculoskeletal tumors of childhood and adolescence. N Engl J Med 29:341–352Google Scholar
  3. 3.
    Nair AP, Bettaswamy G, Jaiswal AK, Garg P, Jaiswal S, Behari S (2011) Ewing’s sarcoma of the orbit with intracranial extension: a rare cause of unilateral proptosis. J Pediatr Neurosci 6(1):36–39PubMedCentralPubMedGoogle Scholar
  4. 4.
    Desai KI, Nadkarni TD, Goel A, Muzumdar DP, Naresh KN, Nair CN (2000) Primary Ewing’s sarcoma of the cranium. Neurosurgery 46:62–68PubMedCrossRefGoogle Scholar
  5. 5.
    Bricha M, Jroundi L, Boujida N, El Hassani MR, Chakir N, Jiddane M (2007) Primary Ewing sarcoma of the skull vault [Article in French]. J Radiol 88:1899–1901PubMedCrossRefGoogle Scholar
  6. 6.
    Garg A, Ahmad FU, Suri A, Mahpatra AK, Mehta VS, Atri S et al (2007) Primary Ewing’s sarcoma of the occipital bone presenting as hydrocephalus and blindness. Pediatr Neurosurg 43:170–173PubMedCrossRefGoogle Scholar
  7. 7.
    Jaurgens HF, Sauer R, Winkelmann W, Gaobel U (1995) Ewing’s sarcoma. In: Peckam M, Pinedo HM, Veronesi U (eds) Oxford textbook of oncology, vol 2. Medical Publications, Oxford, pp 1953–1960Google Scholar
  8. 8.
    Agrawal A, Dulani R, Mahadevan A, Vagaha SJ, Vagha J, Shankar SK (2009) Primary Ewing’s sarcoma of the frontal bone with intracranial extension. J Cancer Res Ther 5(3):208–209PubMedCrossRefGoogle Scholar
  9. 9.
    Chokthaweesak W, Annunziata CC, Alsheikh O, Ng JD, Wilson DJ, Mansoor A, Kaimatchiev V, Weatherhead R, Rootman J, Mannor G, Leelapatranurak K, Korn BS, Kikkawa DO (2001) Primitive neuroectodermal tumor of the orbit in adults: a case series. Opthal Plast Reconstr Surg 27(3):173–179Google Scholar
  10. 10.
    Nair Anup P, Bettaswamy Guruprasad, Jaiswal Awdhesh K, Garg Pallav, Jaiswal Sushila, Behari Sanjay (2011) Ewing’s sarcoma of the orbit with intracranial extension: a rare cause of unilateral proptosis. J Pediatr Neurosci 6(11):36–39PubMedCentralPubMedGoogle Scholar
  11. 11.
    Guzowski M, Tumuluri K, Walker DM, Maloof A (2005) Primary orbital Ewing sarcoma in a middle-aged man. Opthal Plast Reconstr Surg 21(6):449–451CrossRefGoogle Scholar
  12. 12.
    Kano T, Sasaki A, Tomizawa S, Shibasaki T, Tamura M, Ohye C (2009) Primary Ewing’s sarcoma of the orbit: case report. Brain Tumor Pathol 26(2):95–100PubMedCrossRefGoogle Scholar
  13. 13.
    Coley BL, Higinbotham NL, Bowden L (1984) Endothelioma of bone (Ewing’s sarcoma). Ann Surg 128:533–560Google Scholar
  14. 14.
    Jain S, Kapoor G (2010) Chemotherapy in Ewing’s sarcoma. Indian J Orthop 44(4):369–377PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2014

Authors and Affiliations

  • Syed Hassan Abbas Naqvi
    • 1
    • 3
  • Saad Hameed
    • 2
  • Syed Hassan Shiraz Naqvi
    • 1
  • Muhammad Anis Musani
    • 2
  1. 1.Dow Medical CollegeDow University of Health SciencesKarachiPakistan
  2. 2.Sindh Medical CollegeDow University of Health SciencesKarachiPakistan
  3. 3.KarachiPakistan

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