Isolated myxopapillary ependymoma of the fourth ventricle: illustrative case

Abstract

BACKGROUND: A myxopapillary ependymoma (MPE) is a distinctive ependymoma variant that arises exclusively in the conus medullaris or the filum terminale. There are few case reports of intracranial occurrences, with even rarer occurrences in the fourth ventricle. OBSERVATIONS: A 31-year-old female patient presented with difficulty in maintaining her balance and headaches of 3 months' duration. Brain magnetic resonance imaging showed a fourth ventricle mass with obstructive hydrocephalus. Surgical removal of the tumor was performed. The biopsy of the tumor revealed the histological feature of MPE, World Health Organization grade 2. To the authors' knowledge, this is the fourteenth reported case of primary intracranial MPE and the fifth reported case of MPE originating from the fourth ventricle. LESSONS: MPEs are known for their notorious adhesion and infiltration of adjacent neural tissue; therefore, the clinical outcome of fourth ventricle MPEs depends on their relationship with the floor of the fourth ventricle. https://thejns.org/doi/10.3171/CASE24571.