Clival chordoma presenting with isolated unilateral cranial nerve XII palsy: A case report

Abstract

Introduction: Chordomas are rare malignant neoplasms that can originate anywhere along the cerebrospinal axis. However, they are most commonly found in the spine, cranium, and sacrococcygeal region. Chordomas can manifest differently depending on their location and most symptoms are a result of local invasion. We present a rare case of intracranial clival chordoma that manifested as isolated unilateral cranial nerve XII (CN XII) palsy.

Presentation: A 53-year-old male presented to the neurosurgical clinic with headaches, dysarthria, and pharyngeal pain. Neurological examination showed left-sided atrophy of the tongue. MRI scan showed an infiltrative lesion in the clivus which was hypointense on T1 and hyperintense on T2. The lesion was treated surgically however full resection was not achieved. Pathological examination and subsequent immunohistochemical staining confirmed the diagnosis of chordoma.

Discussion: To our knowledge, there have been only two reported cases of clival chordoma that presented with isolated CN XII palsy which manifested clinically as dysarthria and unilateral atrophy of the tongue. This makes our case the third reported case of clival chordoma that presented with isolated CN XII palsy.

Conclusion: We report a rare case of clival chordoma that presented with isolated left CN XII palsy. Physicians should consider clival chordomas in their differential diagnoses when faced with isolated unilateral CN XII palsy. Surgical resection combined with adjuvant radiotherapy remains the preferred treatment protocol.

Keywords: Case report; Chordoma; Cranial nerve XII palsy; Dysarthria; Surgical resection.

Fig. 1

A. T2-weighted MRI, axial view. The arrow shows hyperintense Clival lesion located 3 mm from the anterior end of the clivus, the mass can be seen compressing the left internal carotid artery. (pre-operative MRI). B. T1-weighted MRI, sagittal view. The arrow shows hypointense mass causing severe destruction of the clival cortex, the mass can be seen compressing the anterior aspect of the medulla. (pre-operative MRI).

Fig. 2

T2-weighted MRI, axial view. Signs of surgical intervention on the clivus are noted. The arrow shows residual tumor. (post-operative MRI).

Fig. 3

A. Chordoma (H&E 100×). Prominent myxoid background containing small columns or clusters of bubbly physalipharous cells. B. Chordoma (H&E 400×). Occasional cells with irregular or hyperchromatic nuclei.

Fig. 4

A. Chordoma, Immunohistochemical staining. Arrows show positivity for cytokeratin (CK). B. Chordoma, Immunohistochemical staining. The arrow shows positivity for epithelial membrane antigen (EMA).