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40-Year-Old with Headache and Abducens Palsy
This case features a 40-year-old presenting with headache and abducens palsy, ultimately diagnosed with a clival chordoma.
Imaging overview:
Axial T2 MRI demonstrates a markedly T2 hyperintense midline destructive mass arising from the clivus, with the classic “thumbprint sign” of posterior extension indenting the pons. Sagittal FLAIR confirms the midline clival origin and posterior pontine indentation. Axial T1 fat-saturated post-contrast MRI shows variable enhancement of the mass, characteristic of chordoma.
Clinical insight:
Chordomas are locally aggressive tumors arising from notochordal remnants within bone, presenting as destructive extradural midline lesions. They occur in decreasing frequency at the sacrococcygeal region, skull base, and vertebral body. At the skull base, the clivus is the most common site, and the tumor can extend posteriorly to compress the brainstem, producing the thumbprint sign on imaging. Cranial nerve palsies, particularly abducens palsy due to the proximity of CN VI to the clivus, are a common clinical presentation. Marked T2 hyperintensity reflecting the myxoid matrix of the tumor is a key imaging feature, with variable degrees of enhancement on post-contrast sequences. MRI is essential for characterizing soft tissue and neurovascular involvement for surgical planning.
Case courtesy of Jessica Houk, MD, Duke University Medical Center.