Abstract
Introduction
Neurofibromatosis type 2 (NF2) is characterized by often bilateral vestibular schwannomas (VS) that result in progressive hearing loss and compression of nearby brainstem structures causing cranial nerve palsies. Treatment of these tumors remains challenging, as both surgical removal and expectant management can result in symptom progression. Stereotactic radiosurgery (SRS) has been investigated for the management of NF2-associated VS; however, the role, promises, and pitfalls of this treatment modality remain unclear.
Methods
Ovid MEDLINE, EMBASE, Web of Science, and Cochrane Reviews were searched for studies assessing SRS outcome in NF2-associated VS only. Primary endpoints included tumor control, serviceable hearing, presence of tinnitus, and cranial nerve V and VII symptoms.
Results
A total of 16 studies (589 patients harboring 750 tumors) were analyzed. Clinical tumor control was achieved in 88% of cases (95% CI 80–95%); salvage surgery was needed in 8% (95% CI 4–13%) of cases. Treatment resulted in a worsening of pre-treatment serviceable hearing (OR = 0.26, p < 0.01), increased facial nerve (OR = 1.62, p < 0.01) and trigeminal nerve (OR = 1.42, p = 0.07) impairment. The incidence of vestibular symptoms and hydrocephalus were not consistently reported and thus could not be assessed.
Conclusions
The treatment of NF2-associated VS continues to pose a challenge, as current SRS regimens result in impaired hearing and worse cranial nerve comorbidities, despite achieving high tumor control. It remains unclear if these findings have to be regarded as treatment complications or, rather, continued disease progression.
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Data availability
The datasets generated during and analyzed during the current study are publicly available as they are here summarized and available for repeated statistical analysis, as warranted.
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Anjile An, MPH, was partially supported by the following grant: Clinical and Translational Science Center at Weill Cornell Medical College (1-UL1-TR002384-01).
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All authors contributed to the study conception. UT, AB, JPK, and RR designed this study. Material preparation, data collection and analysis were performed by UT, OM, AA, MET, LSWG, and APDR. In particular, abstract screening and data extraction were carried out by UT and OM; literature search was carried out by APDR, and statistical analysis was carried out by UT, AA, and PC. The first draft of the manuscript was written by UT and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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Tosi, U., Maayan, O., An, A. et al. Stereotactic radiosurgery for vestibular schwannomas in neurofibromatosis type 2 patients: a systematic review and meta-analysis. J Neurooncol 156, 431–441 (2022). https://doi.org/10.1007/s11060-021-03910-8
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DOI: https://doi.org/10.1007/s11060-021-03910-8