Chordomas originate from remnants of the embryonic notochord. The peak age of onset is between 30 to 40 years. Chordomas are benign tumors that grow slowly and have a prolonged clinical course.
Clival chordomas primarily present with symptoms such as headache, visual disturbances, nasal obstruction, and neck pain. Bilateral abducens nerve impairment is a characteristic feature. If the tumor extends into the cerebellopontine angle, symptoms such as hearing loss, tinnitus, and vertigo may occur. Chordomas originating near the nasopharynx tend to protrude into the nasopharyngeal cavity or invade the paranasal sinuses, causing nasal obstruction, headache, and bloody or purulent discharge. Nasopharyngeal symptoms often appear before neurological involvement and warrant close attention.
On CT, chordomas typically present as bone destruction at the lesion site, often accompanied by focal calcifications within the tumor. On MRI, the tumor generally appears as a hyperintense signal on T1-weighted images and as hypointense or isointense on T2-weighted images, with significant contrast enhancement.
The diagnosis of chordoma can be established based on a history of chronic headaches, involvement of multiple cranial nerves, and characteristic imaging findings. In cases where a nasopharyngeal mass is present, a biopsy can confirm the diagnosis. Chordomas need to be differentiated from nasopharyngeal carcinoma.
Treatment usually consists of surgical resection and radiotherapy, although the therapeutic outcomes are often unsatisfactory. The prognosis for chordomas is poor, with a 5-year survival rate of 30% to 50%.