Nasopharyngeal Chordoma

Introduction: Chordoma is a rare malignant bone tumor, originating from embryonic remnants of the notochord. It is more common in males, with the highest incidence between 40 and 60 years, with slow-growing behavior and rarely occurring metastases. They are located mainly in the cephalic extremities and lumbar-sacral. Treatment of its skull base and pharyngeal location is basically surgical, supplemented by radiotherapy.

Objectives: The aim of this study is to report a case of chordoma of the nasopharynx resected by endonasal endoscopic approach at the Hospital Santo Antônio, Brazil.

Resumed Report: A 52-year-old female patient (D. S.), lives in Salvador and presented nasal obstruction for about 1 year. Nasal endoscopy showed the nasopharynx totally occupied by a smooth and regular tumor mass. Computed tomography showed a large, solid, and hypervascular mass obliterating the nasopharynx and magnetic resonance a solid, vascularized tumor, isointense on T1 and hyperintense on T2. The first biopsy of the lesion showed chronic inflammation. Then, endonasal endoscopic surgery was planned and performed with complete excision of the lesion. Pathology showed myxoid tumor and immunohistochemistry confirmed nasopharyngeal chordoma. The patient was referred for oncologic evaluation and remained recurrence-free.

Conclusion: Because of the rarity of this tumor and lack of early specific signs, otolaryngologists tend to underdiagnose this disease, resulting in delay of treatment. Thus, a detailed history associated with nasopharyngolaryngoscopy, imaging, and biopsy tests should be conducted early to confirm the diagnosis and treatment, improving the prognosis for these patients.