Cholesteatoma of the clivus

Abstract

Cholesteatomas (central nervous system epidermoids) can be found intradurally or extradurally in the central nervous system. Extradural intraosseous lesions are most commonly found in the petrous bone. The authors describe a unique case of a clival cholesteatoma in a 64-year-old woman who presented with headaches. No other neurological complaints or physical examination findings were noted. Magnetic resonance imaging showed an expansile lesion centered in the middle portion of the clivus. A large portion of the clivus was eroded. The lesion was explored via a transnasal trans-sphenoidal approach and granular debris was evacuated. The cystic lining was stripped from the surrounding bone, and the bone opening was widely fenestrated. Pathological examination showed keratinous debris with macrophages and an outer lining of benign epithelial tissue consistent with a cholesteatoma (epidermoid cyst). When surgically accessible, these lesions should be excised to prevent a recurrence. If inaccessible, marsupialization may be considered.

Figure 1

MRIs from a 64-year-old woman with headaches showing an erosive lesion in the clivus. T1-weighted (A) axial and (B) sagittal images show a mixed signal intensity lesion in the clivus with a hyperintense central core surrounded by an area of hypointense signal. (C) Post-gadolinium T1-weighted sagittal MRI shows peripheral enhancement.

Figure 2

(A) Material removed from within the cyst is largely composed of layers of anucleate squames. (B) A portion of thin benign epithelial tissue associated with overlying keratinizing squames and an underlying thickened basement membrane is evident. Hematoxylin and eosin stain.