Clival chordoma

Case contributed by Dr Dylan Kurda

Presentation

A 47-year old man with progressive headache, diplopia and sensory deficit (numbness) in his face.

Patient Data

Age: 47 years
Gender: Male
Modality: MRI

There is a large, well defined, T1 isointense mass lesion centred over the clivus, with small high signal foci, which my represent intratumoural haemorrhage. The lesion is T2 hyperintense and shows vivid, but heterogenous, enhancement on post-contrast images. It is invading the sphenoid sinus anteriorly, and the pontine cistern posteriorly, where it also abuts the basilar artery. It invades both cavernous sinuses, more pronounced on the left side with evidence of encasement of the left internal carotid artery (cavernous portion).

Case Discussion

MRI features are likely representing clival chordoma, which was proven on histopathology postoperatively. 

Clival chordoma represent the second commonest site of chordoma (sacrococcygeal location being the first location). Chordomas are uncommon malignant tumours that account for 1% of intracranial tumours and 4% of all primary bone tumours. 

They originate from embryonic remnants of the primitive notochord (earliest fetal axial skeleton, extending from the Rathke's pouch to the coccyx). Since chordomas arise in bone, they are usually extradural and result in local bone destruction. They are locally aggressive, but uncommonly metastasise.

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Case Information

rID: 36795
Case created: 11th May 2015
Last edited: 13th Apr 2016
Inclusion in quiz mode: Included

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