INTRODUCTION: The aim of this study was to analyze and discuss epidemiology, clinical-radiological features, differential diagnosis, histopathologic characteristics, treatment strategies and long-term follow-up results of pediatric vestibular schwannomas treated at a single institution.
METHODS: Medical records of pediatric patients who were operated for vestibular schwannomas in our department between 2000 and 2017 were retrospectively reviewed.
RESULTS: Ten patients (7 girls, 3 boys) with vestibular schwannomas were included. Two patients had neurofibromatosis type 2 syndrome (NF2), while others were sporadic cases. The mean age at diagnosis was 14.9 ± 2.3 years. Major presenting symptoms and signs were hearing loss, tinnitus, headache, imbalance, hemiparesis and facial numbness. Patients had an average of 2.5 ± 1.0 years of symptom duration. In 8 of 10 patients total or near-total tumor resection was achieved. Although the facial nerve was anatomically preserved in all patients, early facial dysfunction occurred in 60%. At the end of an average follow-up of 9.4 ± 5.0 years, 80% of the patients had normal or acceptable facial nerve function (House Brackman grade 1 or 2), whereas 2 patients had permanent facial paralysis (House Brackman grade 5 or 6). Tumor progression and recurrence were observed in two patients.
DISCUSSION AND CONCLUSION: Pediatric vestibular schwannomas are rare tumors and commonly associated with NF2. Expected long-term survival necessitates effective treatment. Microsurgery is a powerful strategy with possibility of total tumor removal and minimal morbidity rates. Radiosurgery can be used particularly in residual or recurrent tumors or those not exceeding 2-3 cm, although long-term results were not well known in children.