Case report Extraskeletal Ewing's sarcoma of the parapharyngeal space

Extraskeletal Ewing's sarcoma (EES) is rarely found in the head and neck region. We report here a case of EES of the parapharyngeal space in a 53-year-old man who presented with blurred vision, dysphagia, hoarseness and right facial numbness. CT examination showed a large, seemingly well-defined soft tissue mass in the right parapharyngeal space with skull base destruction and intracranial extension. The patient showed poor response to chemotherapy and radiotherapy and died 6 months after initial presentation. A review of the literature revealed no previous reports of EES occurring in the parapharyngeal space. Extraskeletal Ewing's sarcoma (EES) is a rare, rapidly growing, round-cell, malignant tumour that can develop in the soft tissues at any location. It was first described by Tefft et al (1) in four patients who presented with paravertebral soft tissue tumours with histological appear- ance resembling Ewing's sarcoma. In a clinicopathological study of 39 cases of EES reported by Angervall and Enzinger (2), the paravertebral region was the most frequent site of involvement, followed by the lower extremity and chest wall. The head and neck region is an unusual primary site for this tumour and, according to Chao et al (3), there were only five out of 118 cases of EES located in the head and neck region. Herein, we report a case of EES occurring in the parapharyngeal space.