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

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.