J Korean Neurosurg Soc.  2025 May;68(3):278-285. 10.3340/jkns.2024.0237.

Management Strategies of Neurofibromatosis Type 2 in Pediatric Patients : Challenges and Emerging Therapies

• Lee JS

• KMID: 2567459
• DOI: http://doi.org/10.3340/jkns.2024.0237

Abstract

Neurofibromatosis type 2 (NF2) is a rare genetic disorder caused by mutations in the NF2 tumor suppressor gene, characterized by bilateral vestibular schwannomas and other central and peripheral nervous system tumors. Pediatric patients often present with more aggressive disease, greater tumor burdens, and increased morbidity compared to adults. Management requires a multidisciplinary approach that balances tumor control with functional preservation. While surgery and radiosurgery remain key treatment options, they carry risks such as hearing loss and malignant transformation of existing tumors. Bevacizumab and emerging therapies like gene therapy show promising therapeutic effects but are limited by variability in efficacy. Comprehensive care, including psychosocial support, is essential to improve clinical outcomes and quality of life for children with NF2.

Keyword

Neurofibromatosis 2; Pediatrics; Neoplasms; Schwannoma; Therapeutics