P13.17 * STEREOTACTIC RADIOSURGERY WITH GAMMA KNIFE FOR NEUROFIBROMATOSIS 2-ASSOCIATED VESTIBULAR SCHWANNOMAS

Abstract

BACKGROUND: It is though that Stereotactic Radiosurgery (SRS) is less effective in achieving local tumor control in Neurofibromatosis 2 (NF2)-related vestibular schwannomas (VSs) compared with sporadic tumors. There is scarce literature on optimal dosing, clinical outcomes and eventual increased risk for malignant transformation among these patients. It is also possible that radiation induced tumors, in patients bearing an abnormality in a tumor suppressor gene, are misinterpreted as part of the natural history of NF2, where new tumors are expected to develop. OBJECTIVE: To evaluate the results of Gamma Knife (GK) Radiosurgery in the management of NF2-related VSs versus the sporadic VSs group treated at the same Center. METHOD: A prospectively maintained clinical database including all patients who underwent SRS for VSs was reviewed, and all subjects fulfilling the Manchester diagnostic criteria for NF2 were identified. Between 1994 and 2012, 35 patients with NF2 underwent SRS for 55 presumed VSs at our institution. The mean age at time of treatment was 30.8 years and the mean follow-up period was 4.3 years (1-14.75 years). The median margin dose used was 12 Gy and a total of 62 treatments were performed. Outcome measures, including imaging progression, hearing preservation, trigeminal and facial nerve function, were analyzed. RESULTS: Regarding tumor control 53.4% remained unchanged in size, 22.4% were smaller and 22.4% showed progression requiring further microsurgical resection or SRS. Hearing worsening occurred in 37% of tumors; 2 patients developed facial neuropathy, one of them bilateral and the other transient; trigeminal neuropathy occurred in 4 patients, one of them with previous mild impairment. No cases of malignant transformation were reported. Compared to the sporadic VSs group, NF2 patients showed lower local tumor control and higher incidence of facial and trigeminal neuropathy. CONCLUSION: NF2-related VSs treated with GK show worse clinical and imaging outcomes compared to the sporadic tumors. Although results do not seem to be as good as for patients with sporadic unilateral tumors, GK radiosurgery, using conventional doses, seems to offer acceptable local tumor control rates (75.8%), indicating that radiosurgery could be considered for primary tumor management in selected patients. In order to confirm if radiation therapy is a predisposing factor for the development of new tumors in these patients, a larger multicenter study needs to be designed.