Purpose: Review the imaging findings of a chordoma and correlate these with the embryologic origin of the lesion.

Case Report: A 26 year old female presenting with one year of bilateral upper extremity paresthesia’s worse with neck hyperflexion (Lhermitte’s phenomenon), upper extremity hyperreflexia, and constant dull neck pain found to have an extradural mass at C3-5 originating from the lateral C4 vertebral body.  Following imaging work-up, the patient underwent preoperative embolization of the left vertebral artery followed by resection, anterior cervical spinal fusion, and adjuvant radiation. 

Imaging Findings: On MR, this extra-dural mass was avidly T2 hyperintense and T1 hypointense with lobular contours arising from the lateral C4 vertebral body filling and expanding the left foramen transversarium.  This mass undermined and uplifted the posterior longitudinal ligament and caused severe canal stenosis displacing the thecal sac posteriorly.  Post contrast images demonstrated ill-defined, minimal linear enhancement of the mass and with patent left vertebral artery. A CT myelogram performed demonstrated an extra-dural expansile lesion without any osseous matrix or intra-tumoral calcification along the posterolateral surface of the C3-5 vertebral bodies. There was significant expansion of the left foramen transversarium and encase of the left vertebral artery without cortical disruption or osseous erosion. 

Summary: Chordoma’s are slow growing tumors which arise from notochordal remnants, which reside in the midline of the neuroaxis from the basio-occiput to the sacrum. These tumors characteristically contain physaliphorous (“bubble bearing”) cells with vacuolated, lobular cytoplasm giving these lesion an expansile, avidly hyperintense T2 appearance with lobular contours. Both MR and CT are often needed to differentiate these lesions from chondrosarcomas which can have a similar MR appearance, but CT can differentiate the chondroid matrix seen in chondrosarcomas versus lytic destruction and resultant erosive calcification seen with chordomas. Historically thought be most common in the sacrum or clivus, chordomas are now known to be relatively equally distributed between the occiput, spine, and sacrum.  This case illustrates the classic imaging appearance of a chordoma regardless of where it originates along the cranio-caudal axis in the spine.

From Clivus to Coccyx and Anywhere in Between, a Case Presentation of a Mid-Cervical Chordoma.

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