Management strategies for vestibular schwannomas in patients with NF2-related schwannomatosis: a systematic review

Abstract

BACKGROUND: NF2-related schwannomatosis is a rare tumor-predisposition syndrome characterized by bilateral vestibular schwannomas (VS) that frequently lead to progressive hearing loss and neurological morbidity. Multi-modality strategies-microsurgery, radiosurgery, radiotherapy, systemic therapy, observation-are employed, but optimal treatment selection remains challenging due to disease heterogeneity, evolving practice patterns, and limited comparative evidence. This study assesses reported management strategies and outcomes for NF2-associated VS. METHODS: A systematic review was performed for studies reporting management strategies and outcomes for NF2-associated VS, including cohort studies and case series describing treatment methods and outcomes. Data collected included treatment modalities, tumor control, hearing outcomes, and facial nerve function. RESULTS: Of 359 records, 32 studies met the inclusion criteria, mostly retrospective case series. Radiosurgery represented the most frequently reported modality, followed by microsurgery. Radiosurgery achieved tumor-control rates ranging of 66%-100%, 5-year control rates of 81%-85%, and hearing preservation of 33%-67%. Microsurgical series generally involved larger tumors, with resection rates of 66%-92%, good facial nerve outcomes from 49.6% to 83.2%, and variable hearing preservation. Radiotherapy demonstrated tumor control of 87%-94%. In bevacizumab cohorts, radiographic response was observed in roughly one-third of tumors, stable disease in about half, and rebound growth after withdrawal in a subset. CONCLUSION: Management of NF2-associated VS remains individualized. Radiosurgery provides tumor control for selected tumors, but contemporary decision-making increasingly weighs long-term hearing goals, systemic options, and concern for radiation-associated malignant progression. Microsurgery prevails for larger or symptomatic lesions. Prospective studies with standardized outcomes and modern treatment comparisons are needed to refine treatment strategies.