Linear accelerator-based stereotactic radiosurgery for bilateral vestibular schwannomas in patients with neurofibromatosis type 2.

OBJECTIVE: Patients with neurofibromatosis Type 2 (NF2) patients typically have bilateral vestibular schwannomas (VS) and are at risk for developing bilateral deafness, bilateral trigeminal, and bilateral facial nerve function loss. Previous reports suggested that treatment outcomes in these patients are worse compared with those for patients with sporadic solitary VS. Very few reports, however, have been published on linear accelerator-based radiosurgery (RS) and stereotactic radiation therapy (SRT) in patients with NF2. In particular, in patients with NF2 who already have unilateral hearing loss, avoidance of hearing loss on the opposite side poses a challenge for RS and SRT. We studied our treatment results in patients with NF2 with bilateral VS, treated with linear accelerator-based RS and SRT.
METHODS: In 204 patients with VS treated with RS or SRT in Amsterdam starting from 1992, we identified 25 patients with NF2 who had bilateral tumors. Indications for treatment were either tumor progression on sequential magnetic resonance imaging scans and/or progressive hearing loss. Mean tumor diameter was 2.5 cm. Stereotactic irradiation was administered to all patients using five noncoplanar arcs with a single isocenter to a dose of 10 to 12.5 Gy in a single fraction or 20 to 25 Gy in five fractions in 1 week prescribed to the 80% isodose encompassing the tumor. On the untreated side, all patients showed hearing loss and eight (32%) had ipsilateral deafness. Five patients were followed for less than 1 year. Of the remaining 20 patients, five had ipsilateral deafness before treatment. Consequently, 15 patients were at risk for treatment-related hearing loss. They showed a mean pure tone average (PTA) of 51 dB (8-112 dB) before treatment. After treatment all patients were assessed at yearly intervals including magnetic resonance imaging and pure tone audiometry.
RESULTS: Median follow-up time was 51 months (12-109 mo). Local tumor control was obtained in all 20 patients, and no treatment-related trigeminal or facial nerve toxicity was observed. Hearing status was assessed yearly after treatment. This assessment revealed that the mean PTA in the 15 hearing patients dropped from 51 to 77 dB (40-120 dB). In six patients (40%) the additional PTA loss ranged from 0 to 15 dB, in another six (40%) it ranged from 15 to 45 dB, and in three of these patients (20%), it was more than 45 dB. No additional hearing loss was observed beyond 36 months after treatment.
CONCLUSION: In this largest series in the literature of linear accelerator-based RS and SRT for VS NF2 patients, excellent local control rates were found with minimal facial and trigeminal nerve toxicity. Although more than 40% of the patients retained their hearing level or lost less than 15 dB of PTA on the irradiated side, preservation of hearing remains a major concern.