Radiosurgery for bilateral neurinomas associated with neurofibromatosis type 2

The clinical course of bilateral acoustic tumors associated with neurofibromatosis (NF2) is generally troublesome, and no definite treatment strategy has been established. Follow-up results of bilateral acoustic tumors after radiosurgery are reported herein.

The current indications for radiosurgery are 1) a growing tumor less than 30 mm in mean diameter, 2) the ipsilateral ear has no serviceable hearing, and 3) there is risk of brain stem compression or brain stem dysfunction. Twenty cases of bilateral acoustic tumors were treated with the gamma knife, including 7 males and 13 females. The mean age was 38.2 years and the mean tumor size 24.4 mm. The tumors were treated with mean maximum and marginal doses of 26.8 Gy and 13.0 Gy, respectively. Among them, 12 patients had profound hearing loss in the ipsilateral (treated) ear, but the other 8 had serviceable hearing.

Tumors treated with radiosurgery showed central necrosis in 60% of the cases at 6 months and in 70% at 9 months after radiosurgery. Thereafter, the tumors often demonstrated slow regression. The rate of tumor shrinkage was 20% at 12 months, 35% at 24 months, and almost 60% at 36 months. At the last follow-up (mean 33.6 months), the tumors demonstrated shrinkage in 50% and tumor control in 100%. The contralateral tumors were stable in 12 (60%) and enlarged in 8 (40%). Preservation of serviceable hearing ipsilaterally was obtained in 33.3%. Deterioration of ipsilateral facial nerve function, either in the natural course or as a complication, occurred in 10%.

Because of good tumor control and tumor shrinkage as well as an acceptable complication rate, radiosurgery should be incorporated in the treatment strategy for bilateral acoustic tumors associated with NF2.