Abstract
Chordomas are rare malignant tumors originating from notochordal remnants that give rise to midline structures of the axial skeleton, including the skull base, mobile spine, and sacrococcyx. Extraosseous soft tissue chordomas near the skull base are extremely rare. We present a case of a 58-year-old Hispanic male with a two-month history of dysphagia, mild odynophagia, difficulty breathing, voice changes, decreased appetite, and weight loss. Imaging revealed a 3.9 cm heterogeneously enhancing retropharyngeal mass. He underwent transoral resection, and pathology confirmed chordoma. He remained disease-free postoperatively until surveillance imaging found recurrence. Over nearly 10 years, the patient underwent three additional surgical excisions for recurrent disease and received proton-beam radiation therapy. He was treated with imatinib, which was stopped due to intolerable adverse effects, followed by dasatinib, which failed to prevent disease progression. He was ultimately switched to afatinib and has since demonstrated relatively stable disease for two years, highlighting the potential of this epidermal growth factor receptor (EGFR) inhibitor as a promising targeted therapy in the context of limited systemic treatment options for chordomas.