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Primary intracranial extraosseous Ewing’s sarcoma

  • Amir Humza Sohail
  • Mohammed Sachal
  • Muhammad Arslan Arif Maan
  • Muhammad Soban
  • Muhammad Salman Khan
  • Muhammad Ehsan Bari
Case Report

Abstract

Introduction

Common sites of occurrence of extraosseous Ewing’s sarcoma include the soft tissues and bones of the lower extremity, 12 paravertebral, and retroperitoneal regions. Primary intracranial Ewing’s sarcoma/pPNET is usually intraparenchymal located 13 when supratentorially, and an extraaxial epidural tumor radiographically mimicking a meningioma is extremely rare.

Case Presentation

A 20-year14 old male presented to the emergency department with a 1-day history of drowsiness, headache, and fever. Neurological exam15 ination revealed decreased muscle strength (4/5) in the left lower limb. Head computed tomography scan showed an epidural 16 space-occupying lesion in the right temporoparietal region, which was assumed to be a meningioma by radiographic criteria. However, the surgical specimen was diagnosed as Ewing’s sarcoma.

Conclusion

Primary intracranial extraosseous Ewing’s sarcoma is a rare condition that may mimic a meningioma on imaging. Physicians must be cognizant of this possibility, particularly in any young individual with a solitary contrast-enhancing dural-based lesion.

Keywords

Central nervous system Brain neoplasm Ewing’s sarcoma Epidural Extraosseous Molecular analysis 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Choudhury KB, Sharma S, Kothari R, Majumder A (2011) Primary extraosseous intracranial Ewing’s sarcoma: case report and literature review. Indian J Med Paediatr Oncol 32:118–121CrossRefGoogle Scholar
  2. 2.
    Kumar V, Singh A, Sharma V, Kumar M (2017) Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: a rare tumor with review of literature. Asian J Neurosurg 12(3):351–357CrossRefGoogle Scholar
  3. 3.
    Singleton S, Koerner C (1991) Lenticular lesions: not always an epidural hematoma. Pediatr Emerg Care 17(4):252–254CrossRefGoogle Scholar
  4. 4.
    Mishra S, Parida D, Senapati S, Patra S. Multifocal Ewing’s sarcoma, presenting radiologically as extradural hematoma. Neurol India 2012; 60(\):361PubMedGoogle Scholar
  5. 5.
    Antunes NL, Lellouch-Tubiana A, Kalifa C, Delattre O, Pierre-Kahn A, Rosenblum MK (2001) Intracranial Ewing sarcoma/‘peripheral’ primitive neuroectodermal tumor of dural origin with molecular genetic confirmation. J Neuro-Oncol 51:51–56CrossRefGoogle Scholar
  6. 6.
    D’Antonio A, Caleo A, Garcia JF, Marsilia GM, De Dominicis G, Boscaino A (2004) Primary peripheral PNET/Ewing’s sarcoma of the dura with FISH analysis. Histopathology 45:651–654CrossRefGoogle Scholar
  7. 7.
    Dedeurwaerdere F, Giannini C, Sciot R, Rubin BP, Perilongo G, Borghi L, Ballotta ML, Cornips E, Demunter A, Maes B, Dei Tos AP (2002) Primary peripheral PNET/Ewing’s sarcoma of the dura: a clinicopathologic entity distinct from central PNET. Mod Pathol 15:673–678CrossRefGoogle Scholar
  8. 8.
    Gupta A, Bansal S, Chaturvedi S (2012) Primary Ewing’s sarcoma of frontoparietal bone with major soft tissue extension: an unusual presentation and review of the literature. Case Rep Pathol 2012:713836PubMedPubMedCentralGoogle Scholar
  9. 9.
    Katayama Y, Kimura S, Watanabe T, Yoshino A, Koshinaga M (1999) Peripheral-type primitive neuroectodermal tumor arising in the tentorium. Case report J Neurosurg 90:141–144PubMedGoogle Scholar
  10. 10.
    Mellai M, Caldera V, Comino A, Fortunato M, Bernucci C, Schiffer D. (2010) PNET/ESFT of the cranial vault: a case report. Clin Neuropathol 29:372–377CrossRefGoogle Scholar
  11. 11.
    Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE (2006) Peripheral primitive neuroectodermal tumor/Ewing’s sarcoma of the craniospinal vault: case reports and review. Hum Pathol 37:845–853CrossRefGoogle Scholar
  12. 12.
    Pekala JS, Gururangan S, Provenzale JM, Mukundan S Jr (2006) Central nervous system extraosseous Ewing sarcoma: radiologic manifestations of this newly defined pathologic entity. AJNR Am J Neuroradiol 27:580–583PubMedGoogle Scholar
  13. 13.
    Papotti M, Abbona G, Pagani A, Monga G, Bussolati G (1998) Primitive neuroectodermal tumor of the meninges: an histological, immuno-histochemical, ultrastructural, and cytogenetic study. Endocr Pathol 9:275–280CrossRefGoogle Scholar
  14. 14.
    Stechschulte SU, Kepes JJ, Holladay FP, McKittrick RJ (1994) Primary meningeal extraosseous Ewing’s sarcoma: case report. Neurosurgery 35:143–147CrossRefGoogle Scholar
  15. 15.
    Tanboon J, Sitthinamsuwan B, Paruang T, Marrano P, Thorner P (2012) Primary intracranial Ewing sarcoma with an unusually aggressive course: a case report and review of the literature. Neuropathology 32(3):293–300CrossRefGoogle Scholar
  16. 16.
    Sorensen PH, Lessnickz SL, Lopez-Terrada D, Liu XF, Triche TJ, Denny CT (1994) A second Ewing's sarcoma translocation, t (21; 22), fuses the EWS gene to another ETS–family transcription factor, ERG. Natural Genetics 6(2):146CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Medical CollegeAga Khan UniversityKarachiPakistan
  2. 2.King Edward Medical UniversityLahorePakistan
  3. 3.Department of RadiologyAga Khan UniversityKarachiPakistan
  4. 4.Department of NeurosurgeryAga Khan UniversityKarachiPakistan

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