Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2

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Object. Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To define better the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions.

Methods. Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml, and the mean tumor margin dose was 15 Gy (range 12–20 Gy).

The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 tumors (36%) regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner—Robertson Class I or II) was preserved in six (43%) of 14 patients, and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House—Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients.

Conclusions. Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.

Article Information

Address reprint requests to: Douglas Kondziolka, M.D., M.Sc., F.R.C.S.(C), Department of Neurological Surgery, Presbyterian University Hospital, Suite B400, 200 Lothrop Street, Pittsburgh, Pennsylvania 15213–2582.

© AANS, except where prohibited by US copyright law.

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Figures

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    Axial MR images with coronal reconstructions used in radiosurgical dose planning for a left-sided acoustic neuroma. Seven isocenters were used to conform the dose to the tumor margins. The 50% and 40% isodose lines are displayed.

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    Serial contrast-enhanced MR images obtained in a 19-year-old woman with NF2 who underwent radiosurgery for a right-sided acoustic neuroma. Upper: Axial images obtained 2 years after radiosurgery (left), 50 months after radiosurgery, demonstrating decreased tumor size (center), and 76 months after radiosurgery with no evidence of right-sided tumor (right). Lower: Coronal images obtained 2 years after radiosurgery (left), 50 months after radiosurgery (center), and 76 months after radiosurgery (right).

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