Nuances in the management of cerebellopontine angle ependymomas: illustrative case.

backgroundEpendymomas are one of the most common pediatric brain tumors. Cerebellopontine angle ependymomas (CPAEs) are a subset of ependymomas that pose unique surgical challenges due to their location and relationship to surrounding neurovasculature, and they are poorly documented in the current literature. This report discusses the unique tenets of these lesions and their implications for successful management. An illustrative case is presented. OBSERVATIONS: A 15-month-old male was found to have a 2021 classification WHO grade 2 ependymoma extending from the fourth ventricle into the cerebellopontine angle. The mass encased cranial nerves V, VII, VIII, IX, X, and XII and both vertebral arteries, and exerted mass effect on the brainstem. The patient underwent staged resections, and gross-total resection (GTR) was achieved. Post-GTR, adjuvant proton therapy was initiated. Postoperative cranial nerve deficits showed partial to full recovery at the 1-year follow-up. Imaging has shown no residual tumor or tumor recurrence. LESSONS: This experience highlights the success of multiple staged resections to maximize resection while preserving as much neurological functional as possible, but many strategies may be used on a case-by-case basis. This case contributes insight to a poorly documented subset of pediatric ependymomas, reinforcing the need for broader reporting of CPAE to guide future management. https://thejns.org/doi/10.3171/CASE25687.