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Updated: Oct 22, 2025

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Primary sinonasal Ewing sarcoma: A case report.

M F Amri1, A Abdullah2, M I Azmi3

  • 1Universiti Kebangsaan Malaysia Medical Centre, Faculty of Medicine, Departments of Pathology, 56000 Kuala Lumpur, Malaysia.

The Malaysian Journal of Pathology
|August 27, 2021
PubMed
Summary
This summary is machine-generated.

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Ewing sarcoma (ES) is a rare sinonasal tumor. Confirming EWSR1 gene rearrangement is crucial for diagnosing this aggressive small round blue cell tumor, especially in unusual locations.

Area of Science:

  • Oncology
  • Pathology
  • Genetics

Background:

  • Ewing sarcoma (ES) is an aggressive skeletal tumor.
  • Head and neck ES is rare and often misdiagnosed as other small round blue cell tumors.
  • This case highlights ES in the sinonasal area.

Observation:

  • A 5-year-old boy presented with nasal blockage, discharge, and epistaxis.
  • Imaging revealed a maxillary sinus mass with necrosis.
  • Initial differentials included lymphoma and rhabdomyosarcoma.

Findings:

  • Histology showed small round blue cells positive for CD99, NSE, and vimentin.
  • Muscle and lymphoid markers were negative.
  • Fluorescence in-situ hybridization confirmed EWSR1 gene rearrangement, diagnosing ES.

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Implications:

  • Sinonasal ES is rare with overlapping features with other small round blue cell tumors.
  • EWSR1 gene translocation testing is vital for diagnosing ES.
  • Accurate diagnosis is critical for appropriate treatment of sinonasal ES.