Pediatric Sinonasal Ewing Sarcoma

Ewing sarcoma is a malignant tumor usually involving long bones of the extremities. Extraskeletal Ewing sarcoma is less common, involving paravertebral region, chest wall, soft tissue of lower extremities and rarely head and neck region. Ewing sarcoma from the sinonasal cavity is extremely rare. Here we present a sinonasal Ewing sarcoma centered at left anterior ethmoidal air cells from an eleven-year-old girl with EWSR1 rearrangement on FISH and EWSR1::FLI1 gene fusion on nextgeneration sequencing test.