An unusual case of oropharyngeal chordoma

RATIONALE Chordomas are rare malignant neoplasms derived from incomplete regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that develop in an atypical position are called ectopic chordomas, such as oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases have been reported to date. Preoperative diagnosis is challenging, and an accurate diagnosis thus is based on postoperative pathologic examination findings and immunohistochemistry. Although surgical therapy and radiotherapy is performed in some patients, the 5-year survival rate is low. Increasingly more studies of chordomas have been based on molecular biology to increase the survival rate, and targeted therapy could be a new therapy in the future. PATIENT CONCERNS The patient presented with a left oropharyngeal mass that had begun slowly enlarging 1 year previously. He reported a foreign body sensation and dysphonia during this time period. DIAGNOSES The patient was initially diagnosed with a neurogenic tumor. Routine postoperative pathology showed that the mass was consistent with a chordoma. INTERVENTION Mass resection was performed. OUTCOME One year after the initial surgery, magnetic resonance imaging revealed block signal images at the left retropharyngeal space and clivus. The patient developed recurrence of the OC. LESSONS Surgical resection is the mainstay of treatment for OC, and postoperative adjuvant radiotherapy is also important. An understanding of the unusual case described in this report may be helpful in diagnosing OC, and development of targeted therapy may help clinicians to provide novel treatment for patients with OC.