A 47-year old man with progressive headache, diplopia and sensory deficit (numbness) in his face.
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There is a large, well defined, T1 isointense mass lesion centered over the clivus, with small high signal foci, which my represent intratumoral hemorrhage. 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).
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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 tumors that account for 1% of intracranial tumors and 4% of all primary bone tumors.
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 metastasize.
- 1. Harbour JW, Lawton MT, Criscuolo GR et-al. Clivus chordoma: a report of 12 recent cases and review of the literature. Skull Base Surg. 2011;1 (4): 200-6. Free text at pubmed - Pubmed citation
- 2. Erdem E, Angtuaco EC, Van Hemert R et-al. Comprehensive review of intracranial chordoma. Radiographics. 2003;23 (4): 995-1009. doi:10.1148/rg.234025176 - Pubmed citation
- 3. Murphey MD, Andrews CL, Flemming DJ et-al. From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 1996;16 (5): 1131-58. doi:10.1148/radiographics.16.5.8888395 - Pubmed citation
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