Background: Extraskeletal Ewing's sarcoma [EES] is an unusual aggressive malignant tumor that is neuroectodermal cell in origin. Presentation in the scapula region is extremely rare. We herein present a case report and literature review of EES of the scapula.

Case Report: An 18-year-old Thai female patient presented with a 1-year history of a gradual swelling on her right scapula that had rapidly progressed during the past 2 months. The patient was
subsequently diagnosed with EES with pulmonary metastases. She received multi-agent chemotherapy and underwent debulking surgery. Despite this aggressive treatment, her clinical condition worsened and she died of pulmonary metastasis.

Conclusion: EES of the scapula is rare, and knowledge regarding therapy for this condition is still limited. Despite multimodal treatment approaches including surgery, multi-agent chemotherapy, and radiation, the prognosis remains poor.

Keywords: Extraskeletal Ewing's Sarcoma, Scapula, Bone cancer