(869) Pediatric-Onset Intratemporal Facial Nerve Tumors: Presentation, Genetics, and Facial Outcomes by Management Strategy

Introduction: Pediatric intratemporal facial nerve (FN) tumors are rare. Adult paradigms often emphasize radiotherapy/radiosurgery or definitive resection, but pediatric considerations differ. We describe presentation, genetics, management, and facial outcomes.

Methods: Retrospective case series at a pediatric otology/neurotology center. Patients presenting ≤18 years with intratemporal CN VII tumors were included. We abstracted diagnosis (pathology vs radiographic), NF2 testing, involved segment, management strategy, and House-Brackmann (HB) grade at last follow-up.

Results: Seven patients met criteria (median age 13.9 years). Six tumors were consistent with schwannoma and one with meningioma; two were diagnosed radiographically. NF2: 2 positive, 2 negative, 1 indeterminate, 1 unknown. Most lesions involved the labyrinthine/geniculate and/or IAC segments; one involved the stylomastoid foramen. Management: microsurgery 4/7 (including middle ear/mastoid FN decompression and limited resection/biopsy; one translabyrinthine resection), radiotherapy 1/7, observation 2/7. Median follow-up 28 months. Facial outcomes were available for 6/7: 5/6 had HB III or better (3 HB II or better); 1 NF2 patient had persistent HB IV and underwent facial reanimation.

Conclusions: Pediatric-onset intratemporal FN tumors may reflect syndromic disease (e.g., NF2) and show heterogeneous anatomic involvement. Observation is reasonable for stable disease; targeted decompression/limited resection can preserve function, and radiotherapy should be weighed carefully. Most patients achieved HB III or better at last follow-up; long-term surveillance is essential. Early MRI and genetics may guide surveillance and counseling.