Characteristics and Outcomes of Pediatric Lateral Skull Base Tumors: 15 Years of Experience

Introduction: Surgical management of lateral skull base tumors requires special considerations in the pediatric population. The deleterious consequences of cranial nerve morbidity (i.e., physical, emotional, social) must be weighed against the advantages of achieving gross total tumor resection. In the adult population, there has been a trend toward more conservative tumor resection in favor of cranial nerve preservation; ideal management of pediatric tumors is unclear and poorly studied. In this series, we report our 15-year experience with pediatric lateral skull base neoplasms, as well as treatment patterns and outcomes at a large tertiary referral center.

Methods: Medical records of pediatric patients (<18 years of age) with lateral skull base neoplasms between 2004 and 2020 were reviewed for demographic data, imaging, and audiometric and surgical outcomes.

Results: A total of 46 ears (34 patients) were included in analysis. Fifty-three percent (n = 18) of patients were female, 57% (n = 26) of tumors were left-sided, and 34% (n = 11) of patients had neurofibromatosis type 2 (NF2). The majority of pathology identified included acoustic neuroma (65%, n = 30), followed by lower cranial nerve schwannoma (11%, n = 5), facial nerve schwannoma (9%, n = 4), and trigeminal schwannoma (2.2%, n = 1). Median age of diagnosis was 12.5 years, and the most common presenting symptom was hearing loss. Fifty-seven percent (n = 26) of ears underwent surgical resection at a median age of 13.5 years (interquartile range]: 9.0–17.8 years). Mean tumor diameter in greatest dimension at time of diagnosis was 14.9 mm. Translabyrinthine approach was the most common approach used (n = 10, 38%) followed by retrosigmoid (n = 6, 23%), middle fossa (n = 4, 15%) and infratemporal fossa (n = 4, 15%). The majority of patients achieved gross total resection (n = 18, 69%). Overall, 15 ears (57.7%) required reoperation or additional treatment, of which 53% (n = 8 ears) had NF2. Six (23.1%) ears required reoperation while nine ears (35%) required chemotherapy (n = 6 ears) radiation (n = 1 ear), or chemoradiation (n = 2 ears) Of the NF2 ears undergoing treatment, two required re-operation (18 vs. 27% non-NF2) and six necessitated chemotherapy and/or radiation (55 vs. 20% non-NF2).

Conclusion: Surgical approach and regrowth intervention require careful consideration in the pediatric population, particularly for patients with NF2. While there is a trend toward observation in the management of lateral skull base tumors, a significant proportion of patients in our cohort required reoperation or additional therapy. Of the NF2 patients with regrowth, there was a trend toward nonsurgical management.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021