Clinical features and management of intracranial subependymomas in children

Subependymoma is a rare low-grade glioma of the central nervous system that occurs most commonly in middle-aged and elderly men and rarely in children. Only a few paediatric patients with subependymomas have been reported. The authors retrospectively analysed five paediatric patients (4 males and 1 female; mean age 8.6 years; age range 5–13 years) at a single institute from July 1998 to April 2009 and summarised the clinical characteristics and management of paediatric intracranial subependymoma. The most common symptom in these five paediatric patients with subependymoma was intracranial hypertension. The tumours were located in the fourth ventricle in two patients, in the fourth ventricle with extension to the cerebellopontine angle (CPA) in one patient; in the right CPA exclusively in one patient, and intraparenchymally in the left parietal lobe in one patient, the latter two of which are rare locations for subependymoma. Surgery was performed on all five patients. The surgical approach was selected as appropriate for the tumor location. Total resection was achieved in three patients, and subtotal resection in two. All five patients had good outcomes without recurrence. We conclude that surgery is the optimal therapy for paediatric patients with intracranial subependymoma.