Advances in surgical techniques for resection of childhood cerebellopontine angle ependymomas are key to survival

Childhood cerebellopontine angle (CPA) ependymoma is an uncommon anatomical variant of posterior fossa ependymoma. In infants and young children, the tumor often goes undetected until it causes hydrocephalus. As CPA ependymomas grow, they distort the anatomy and encase cranial nerves and vessels, thereby making resection a formidable surgical challenge. The purpose of this paper is to describe the surgical technique used to achieve gross total resection (GTR) of CPA ependymomas and demonstrate improved survival in these patients. Surgical techniques used for GTR in 45 patients with CPA ependymoma treated from 1997 to 2008 are described. Results of those procedures are compared with data from 11 patients who previously underwent surgical resection (1985-1995). We achieved GTR in 43 (95.6%) patients and near-total resection in two (4.4%); the probability of progression-free survival was 53.8%, and that of overall survival was 64%. Our novel surgical techniques greatly improve central nervous system function and survival among pediatric patients with CPA ependymoma.