We present an unusual case of chordoma arising entirely from the lateral skull base with imaging features suggestive of a paraganglioma. Clinical history, management, histopathology, and imaging characteristics are described, including a review of gallium-dotate PET scanning somatostatinreceptor-positive tumors. We further provide a review of management options, including a summary of our approach with surgical biopsy via retrosigmoid and resection via transtemporal approaches. Based on radiologic characteristics and location, lateral skull base chordoma may arise with isolated lateral skull base involvement and has the potential to be misidentified as a glomus jugulare on initial workup.
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