E-ISSN 2602-4837
The Turkish Journal of Ear Nose and Throat [Tr-ENT]
Tr-ENT. 2018; 28(3): 116-125 | DOI: 10.5606/Tr-ENT.2018.57966

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

İbrahim Başar1, Şahin Hanalioğlu2, Fırat Narin3, Burçak Bilginer4
1Department of Neurosurgery, Siirt State Hospital, Siirt, Turkey
2Department of Neurosurgery, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
3Department of Neurosurgery, Van Regional Training and Research Hospital, Van, Turkey
4Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey

INTRODUCTION: 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.
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.
DISCUSSION AND 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

İbrahim Başar, Şahin Hanalioğlu, Fırat Narin, Burçak Bilginer. Pediatric vestibular schwannomas: Evaluation of clinical features, treatment strategies and long-term results of 10 cases. Tr-ENT. 2018; 28(3): 116-125

Sorumlu Yazar: Şahin Hanalioğlu, Türkiye
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