Chordoma - clivus

Case contributed by Dr Naim K

Presentation

Severe headaches and dysarthria.

Patient Data

Age: 45 years
Gender: Female

Cervical spine

MRI

Straightening of the cervical spine is seen indicating severe muscle spasm.

Vertebral bodies' heights and disk spaces are preserved.

C5-C6: Left posterior lateral disk protrusion is seen indenting thecal sac anteriorly and causing narrowing of the left nerve root exit canal without compressing the spinal cord.

C6-C7: Large left posterior lateral disk protrusion is seen compressing the left anterior lateral aspect of the spinal cord and compressing the left nerve root at its exit canal significantly.

The rest of the cervical intervertebral disks appears normal without evidence of focal disk protrusion or extrusion.

NB:

There is a lobulated soft tissue mass lesion arising from the right posterior inferior aspect of the clivus measuring about 3.2 cm extending posteriorly into the posterior cranial fossa compressing the medulla oblongata and the cervical medullary junction posteriorly without infiltrating it.

The findings are consistent most likely with chordoma.

Brain

MRI

There is evidence of a large lobulated heterogeneously enhancing soft tissue lesion arising from the clivus growing posteriorly and pushing the medulla oblongata posteriorly. This lesion is measuring about 3.5 x 3 cm. This lesion is isointense on T1 with hyperintense areas presenting hemorrhage. Hyperintense on T2WI. It presents most likely to be Chordoma, and less likely to present meningioma. 

There is evidence of early pressure upon the flow of CSF through the foramen of Magendie resulting in early and minimal lateral ventricles dilatation.

There is no evidence of any other focal lesion in the brain.

Case Discussion

Chordomas are uncommon malignant tumors of the axial skeleton, and the spheno-occipital location is the second most common to be involved after sacrococcygeal location. Vertebral body involvement is the least common. Chordomas originate from the bone and result in local bone destruction which is best evaluated by CT imaging.

MRI allows confident preoperative diagnosis. This case illustrates characteristic features of Chordoma which was confirmed on biopsy.

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