Pediatric vestibular schwannomas: Evaluation of clinical features, treatment strategies and long-term results of 10 cases

Authors : Ibrahim Başar, Şahin Hanalioğlu, Fırat Narin, Burçak Bilginer

Pages : 116-125

View : 25 | Download : 6

Publication Date : 2018-09-20

Article Type : Research

Abstract :Objectives: This study aims to analyze and discuss the epidemiology, clinical-radiological features, differential diagnosis, histopathological characteristics, treatment strategies and long-term follow-up results of pediatric vestibular schwannomas VSs treated at a single institution. Patients and Methods: Medical records of 10 pediatric patients 3 males, 7 females; mean age 14.9±2.3 years; range 11 to 18 years who were operated for VSs in our department between January 2000 and December 2017 were retrospectively reviewed. The prognostic variables were age, gender, neurological examination at the time of application, radiologic findings, localization, associated factors, amount of resection total [100%], near total [90-100%] or subtotal [<90%] resection , histopathological grading and adjuvant treatment. Results: Two patients had neurofibromatosis type 2 NF2 , while others were sporadic cases. 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 eight 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 patients had normal or acceptable facial nerve function House-Brackmann grade I or II , whereas two patients had permanent facial paralysis House-Brackmann grade V or VI . Tumor progression and recurrence were observed in two patients. Conclusion: Pediatric VSs are rare tumors 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 are not well known in children.
Keywords : Cerebellopontine angle, microsurgery, pediatric, schwannoma, tumor, vestibular