Practical Approach in Management of Extraosseous Ewing's Sarcoma of Head and Neck: A Case Series and Review of literature.

Extraosseous Ewing's Sarcoma (EES) is a high grade rare malignancy from Ewing's family tumors (EFTs) occurring in soft tissues. Diagnosis of EES relies on a constellation of features, including round cell morphology, characteristic immunohistochemistry (such as CD99, FLI-1 and NKX2.2 positivity), and pathognomic molecular abnormalities of t(11;22)(q24;q12). Multimodality treatment has improved the prognosis and clinical outcome in EFTs. Due to its rarity, the current recommendation to treat EES is based on Skeletal Ewing's Sarcoma (SES) guidelines. However, achieving clear surgical margins in the Head and Neck region is often challenging due to complex anatomy and close proximity to critical structures, placing patients at risk of loco-regional recurrence in the absence of adjuvant therapy. The literature on head and neck EES is scarce, consisting of a few retrospective case series and case reports. Herein, we describe the characteristic clinico-pathological features and treatment of three EES patients with primaries from Parotid, Nasal cavity/ Nasopharynx and Oropharynx, with a comprehensive review of the literature. Keywords: Ewing's Family of tumors, Extraosseous Ewing's sarcoma of head and neck.