E-ISSN 2602-4837
The Turkish Journal of Ear Nose and Throat Pediatric vestibular schwannomas: evaluation of clinical features, treatment strategies and long-term results of 10 cases [Tr-ENT]
Tr-ENT. Ahead of Print: ENT-57966 | 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
1Neurosurgery Clinic, Siirt State Hospital, Siirt, Turkey
2Department of Neurosurgery, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Ankara, Turkey
3Neurosurgery Clinic, Van Regional Training and Research Hospital, Van, Turkey
4Department of Neurosurgery, Hacettepe University School of Medicine, Ankara, Turkey

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.

Keywords: schwannoma, pediatric, vestibular, cerebellopontine angle, tumor, microsurgery



Corresponding Author: Şahin Hanalioğlu, Türkiye
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