Primary Nasopharyngeal Chordoma: A Rare Tumor with Diagnostic and Therapeutic Challenges

Abstract Chordomas are rare, slow-growing tumors arising from notochordal remnants, typically seen in the sacrococcygeal and skull base regions. Primary nasopharyngeal chordoma is an exceedingly rare entity. We report a case of a 60-year-old male who presented with right nostril obstruction and meal-triggered snoring. Imaging revealed a large fludeoxyglucose-avid mass in the nasopharynx with extensive local invasion. Histopathology confirmed chordoma with classic physaliphorous cells and positive immunohistochemistry for cytokeratin, epithelial membrane antigen, S100, and Brachyury. The patient was treated with intensity-modulated radiotherapy (IMRT), but was lost to follow-up posttreatment. This case highlights diagnostic and therapeutic challenges in managing rare skull base chordomas.