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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.
Background:
Ewing sarcoma (ES) is an aggressive tumour which is typically skeletal in origin. ES involving the head and neck region is uncommon and can be easily confused with other small round blue cell tumours. We herein present a rare case of ES involving the sinonasal area.
Case Presentation:
A 5-year-old Somalian boy with no known medical illness presented with progressive nasal blockage associated with clear nasal discharge and intermittent spontaneous epistaxis for three months. CT paranasal sinus and neck region revealed poorly enhancing expansile mass in the right maxillary sinus with areas of necrosis within. Initial radiological differential diagnoses were lymphoma and rhabdomyosarcoma. The mass was biopsied and histologically showed diffuse sheets of small round blue cells that was positive to CD99, NSE and vimentin. The muscle and lymphoid markers were negative. Fluorescence in-situ hybridisation (FISH) study revealed the presence of EWSR1 gene rearrangement thus diagnosis of ES was rendered.
Conclusions:
ES of sinonasal tract is a rare entity and its pathological features significantly overlap with others small round blue cells tumour. Demonstration of EWSR1 gene translocation is recommended for the diagnosis of ES particularly at uncommon sites.
