Extraosseous Ewing’s Sarcoma of the Parapharyngeal Space - A Rare Entity - with Review of Literature

INTRODUCTION: Extraosseous Ewing’s sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing’s sarcoma of the parapharyngeal space is even rarer. To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature. CASE REPORT: We report a rare case of EES of the parapharyngeal space in an 8-year-old girl. She presented with complaints of earache, difficulty in breathing and swallowing and bleeding from the mouth. Investigations revealed a large parapharyngeal mass causing narrowing of the nasopharyngeal and oropharyngeal airway with skeletal and lung metastasis. Biopsy from the parapharyngeal mass was suggestive of malignant small round cell tumor. The patient was treated with chemotherapy and radiotherapy, but developed brain metastasis and succumbed to disease approximately 1 year after diagnosis. Herein, we describe the characteristic clinicopathological features and treatment with a comprehensive review of the literature. CONCLUSION: EES in this unusual location behaves aggressively, with a high rate of recurrence and distant metastasis. Aggressive multimodal treatment comprising of multi-agent chemotherapy, surgical resection if feasible, and radiotherapy should be considered.