Adamantinoma-like Ewing sarcoma of the parotid gland: A case report.

Adamantinoma-like Ewing sarcoma (ALES) of the parotid gland is an exceptionally rare neoplasm. We report the case of a 57-year-old male with a history of p16-positive tonsillar squamous cell carcinoma who presented 2 years after remission with excessive mucus production and neck stiffness. Imaging identified a left parotid mass, and diagnosis was confirmed through surgical pathology, with molecular testing revealing a Fused in Sarcoma-Fifth Ewing Variant (FUS-FEV) fusion gene. Treatment included radical parotidectomy, adjuvant external beam radiation, and systemic chemotherapy following the Ewing sarcoma paradigm. Despite initial response, the patient experienced significant toxicities and eventually succumbed to disease-related complications. This case illustrates the challenges in diagnosing and managing rare parotid malignancies and highlights the importance of multidisciplinary care.