Purpose or Case Report: We present a case of an 8 year old female who presented with recurrent epistaxis. An endoscopy with cauterization was performed and an adenoidal mass was found. A subsequent CT showed a well-circumscribed, hypoattenuating mass within the nasopharynx. The MRI showed a heterogeneous, T2 hyperintense lesion, with predominantly hypointense T1 signal relative to muscle, and with heterogeneous contrast enhancement. An excisional biopsy was performed, and the lesion was found to be an extraosseous chordoma. The goal of this case report is to discuss the characteristics of this relatively rare entity that is a differential consideration for nasopharyngeal mass lesions.

Chordomas are a rare type of malignant neoplasm that predominantly form in the sacrococcygeal and spheno-occipital regions. They arise from embryonic remnants of the primitive notochord, which is a primitive cell line that develops around the skull base and vertebral column. Chordomas are slow-growing, locally invasive tumors that have a low propensity for distant metastasis. They can occur in any age but are seen more commonly in adults with a peak prevalence in the 4th decade of life.

Intracranial chordomas account for 1% of all intracranial tumors. They tend to have high mortality rates due to tumor recurrence and close relation to critical structures at the skull base, such as the optic system, cavernous sinus, carotid arteries, and brainstem. Extraosseous chordomas are a rare subtype, and due to the lack of classic lytic bone changes, they can be difficult to diagnose. Extraosseous chordomas of the nasopharynx can mimic many other common tumors of the nasopharyngeal region, including non-Hodgkin lymphoma, nasopharyngeal carcinoma, and Tornwaldt cysts. When a clival sinus tract is in view, however, extraosseous chordoma should jump towards the top of the differential.
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