Primary Ewing sarcoma of the parotid gland: Case report.

Ewing sarcoma (ES) is a rare malignancy typically involving the skeletal system, but it can present in extraskeletal sites (EES). Primary Ewing sarcoma of the parotid gland is extremely rare, with few cases reported. We present a case of a 24-year-old male with a rapidly growing left parotid mass diagnosed as EES through immunohistochemistry and confirmed by molecular testing. The patient received neoadjuvant chemotherapy, leading to significant tumor reduction, followed by a radical parotidectomy. Histopathology showed residual tumor with over 90 % necrosis. The patient remains disease-free under active surveillance. The rarity of Ewing sarcoma in the parotid gland presents diagnostic challenges. This case emphasizes the importance of molecular testing in establishing the diagnosis and highlights the successful multimodal treatment approach combining chemotherapy and surgery. Literature on EES of the head and neck remains limited, and treatment guidelines continue to evolve.