Primary Chordoma in the Nasal Cavity and Nasopharynx: CT and MR Imaging Findings

BACKGROUND AND PURPOSE: Primary chordoma in the nasal cavity and nasopharynx is an extremely rare tumor in the extraosseous axial skeleton. Unlike intracranial chordomas, lesions in these sites primarily present as a soft tissue mass without involvement of the skull base bone (clivus), so the preoperative diagnosis of the tumor is possibly difficult. Here, we reviewed the imaging features of 5 cases of chordomas in the nasal cavity and nasopharynx that resulted in successful diagnosis and differential diagnosis of this rare tumor.

MATERIALS AND METHODS: We retrospectively studied 5 patients with histologically proven chordomas in the nasal cavity and nasopharynx. The lesion features of CT and MR imaging were reviewed, with emphasis on the size, shape, location, margin, calcification, CT attenuation characteristics, signal intensity, and degree of MR imaging enhancement.

RESULTS: Expansible and lobular soft tissue masses were mainly present, with irregular intratumor calcification in all 5 cases on CT examination. MR imaging revealed a well-defined tumor with heterogeneous signal intensity in 4 patients, whereas homogeneous signal intensity in 1 patient was present on all pulse sequences. Four cases of nasopharyngeal mass showed mild to moderate heterogenous enhancement. Intratumor septa could be seen in 2 cases.

CONCLUSIONS: Although no imaging features are pathognomonic, primary chordomas without skull base (clivus) bony changes in the nasal cavity and nasopharynx have some CT and MR imaging findings that are suggestive of diagnosis. The differential diagnosis of the soft tissue mass should be limited to these sites.