Paediatric intradural cerebellopontine angle chordoma mimicking meningioma

Abstract

Chordomas are rare, notochord-derived neoplasms, most commonly affecting the sacrum and clivus, and exceedingly rare in the cerebellopontine angle (CPA), especially in children. This report describes a 10-year-old male presenting with hearing loss, giddiness, vomiting, and left-sided facial palsy, who was found to have a CPA chordoma that was SMARCB1-deficient, confirmed via histopathology. The clinical presentation and management are discussed, along with a review of the scant global literature.