Chordoma

Case contributed by Prashant Kandel
Diagnosis certain

Presentation

Neck pain, double vision and slurring of speech.

Patient Data

Age: 55 years
Gender: Male
ct

NCCT shows midline, expansile, lytic lesion of the clivus extending towards the right basi-occiput, petro-clival fissure, occipital condyle as well as part of petrous bone.

mri

There is an ill-defined, midline, T1 intermediate to low/ T2 intermediate to slightly high/ FLAIR intermediate signal intensity lesion involving and arising from the clivus associated with its expansion. There are few, tiny, foci of T1 high signal intensities within the lesion representing hemorrhage/ mucus. No any distinct calcific foci. There is no significant blooming artefact on SWI. There is no diffusion restriction of the lesion on DWI/ ADC. Post contrast study shows homogenous enhancement.

Supero-anteriorly, the lesion is extending to protrude into the sphenoid sinus with loss of interface. Supero-posteriorly, the lesion is involving the dorsum sellae with indistinct interface with the posterior pituitary.

Laterally on the right side, it has involved the cavernous sinus and Meckel's cave with encasement of the right internal carotid artery (ICA) causing its luminal narrowing. It has completely encased the right trigeminal nerve (CN V). Right Abducens nerve (CN VI) is also encased by the lesion. Laterally on the left side, its abutting the left ICA with loss of interface.

Infero-posteriorly on the right side, its extending along the right basi-occiput portion of clivus involving the right petro-clival fissure, occipital condyle as well as part of petrous bone. Its soft tissue component is seen extending through the right cerebello-pontine cistern infiltrating into the right cerebellar hemisphere. Its abutting the right facial nerve (CN VII) with maintained interface. It has also involved the right jugular fossa causing encasement and luminal narrowing of the right internal jugular vein (IJV) associated with compression and dilatation of right transverse sinus with loss of interface. There is also encasement of right glossopharyngeal (CN IX), vagus (CN X) and accessory nerves (CN XI). Right hypoglossal nerve (CN XII) is also encased by the lesion within the hypoglossal canal.

Infero-posteriorly on the left side, its extending along basi-occiput portion of clivus. Posteriorly in the midline, its compressing the pre-pontine cistern. Inferiorly, the lesion has reached upto the tip of dens, however, there is no altered signal of its bone marrow.

pathology

Histopathology report of the biopsy taken from lesion confirmed the diagnosis of clival chordoma.

Case Discussion

The midline, expansile, homogenously enhancing lesion arising from the clivus suggests the most likely diagnosis of clival chordoma. This was later confirmed by the histopathology report.

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