Tumor control and hearing preservation after Gamma Knife radiosurgery for vestibular schwannomas in neurofibromatosis type 2 (original) (raw)
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Journal of Neurosurgery, 2017
OBJECTIVENeurofibromatosis Type 2 (NF2) is a tumor syndrome characterized by an autosomal dominant pattern of inheritance. The hallmark of NF2 is the development of bilateral vestibular schwannomas (VSs), generally by 30 years of age. One of the first-line treatment options for small to medium-large VSs is radiosurgery. Although radiosurgery shows excellent results in sporadic VS, its use in NF2-related VS is still a topic of dispute. The aim of this study was to evaluate long-term tumor control, hearing preservation rates, and factors influencing outcome of optimally dosed, contemporary Gamma Knife radiosurgery (GKRS) for growing VSs in patients with NF2 and compare the findings to data obtained in patients with sporadic VS also treated by means of GKRS.METHODSThe authors performed a retrospective analysis of 47 growing VSs in 34 NF2 patients who underwent GKRS treatment performed with either the Model C or Perfexion Leksell Gamma Knife, with a median margin dose of 11 Gy. Actuaria...
Neurosurgery, 2009
OBJECTIVE The aim of this study was to evaluate long-term hearing preservation after gamma knife radiosurgery (GKS) for vestibular schwannomas in patients with initially normal or subnormal hearing (Gardner-Robertson Class 1) and to determine the predictive factors for functional hearing preservation. METHODS Since July 1992, more than 2053 vestibular schwannomas have been treated by GKS and followed at the Timone University Hospital, Marseille. A minimum of 3 years of follow-up (range, 3–11 years; median, 48 months) is available for 74 patients (without neurofibromatosis Type 2 or previous surgery) with Gardner-Robertson Class 1 hearing. RESULTS The average age of the patients was 47.5 years (range, 17–76 years). The number of tumors in Koos Stage I was 8, the average number in Stage II was 21, the average number in Stage III was 43, and the average number in Stage IV was 2. The median number of isocenters was 8 (range, 2–45), and the median marginal dose was 12 Gy (range, 9–13 Gy)...
Journal of the Medical Association of Thailand = Chotmaihet thangphaet, 2004
To evaluate the rates of tumor control and useful hearing preservation in patients with bilateral vestibular schwannomas (VSs) associated with neurofibromatosis type 2 (NF-2) treated with fractionated stereotactic radiotherapy (FSRT). From August 1998--December 2002 there were 5 patients with NF-2 who underwent FSRT (Linac-based system) for bilateral CP angle tumors. Median age was 28 (18-47) years. Median tumor volume was 5.4 (2.2-9.4) cc. Eight lesions received a marginal dose of 44.2-59.9 (median = 46.2) Gy in 25-33 fractions. The other 2 lesions received 4.4 and 4.9 Gy/fraction for 6 fractions in 3 and 2 weeks. Median follow-up was 19 (14-44) months. Radiographic and clinical tumor control rate was 90%. One lesion progressed at 7 months after FSRT and was completely resected Of the 5 lesions with Gardner-Robertson class I-II hearing before FSRT 2 (40%) retained useful hearing at the last follow-up. One patient had left facial spasm at 10 months after FSRT which gradually improve...
Hearing and facial function outcomes for neurofibromatosis 2 clinical trials
Neurology, 2013
Objectives: Vestibular schwannomas are the hallmark of neurofibromatosis 2 (NF2), occurring in .95% of patients. These tumors develop on the vestibulocochlear nerve and are associated with significant morbidity due to hearing loss, tinnitus, imbalance, facial weakness, and risk of early mortality from brainstem compression. Although hearing loss and facial weakness have been identified as important functional outcomes for patients with NF2, there is a lack of consensus regarding appropriate endpoints in clinical trials. Methods: The functional outcomes group reviewed existing endpoints for hearing and facial function and developed consensus recommendations for response evaluation in NF2 clinical trials. Results: For hearing endpoints, the functional group endorsed the use of maximum word recognition score as a primary endpoint, with the 95% critical difference as primary hearing outcomes. The group recommended use of the scaled measurement of improvement in lip excursion (SMILE) system for studies of facial function. Conclusions: These recommendations are intended to provide researchers with a common set of endpoints for use in clinical trials of patients with NF2. The use of common endpoints should improve the quality of clinical trials and foster comparison among studies for hearing loss and facial weakness. Neurology ® 2013;81 (Suppl 1):S25-S32 GLOSSARY AAO-HNS 5 American Academy of Otolaryngology-Head and Neck Surgery; HB 5 House-Brackmann; HR 5 hearing response; NF2 5 neurofibromatosis 2; PTA 5 pure-tone average; REiNS 5 Response Evaluation in Neurofibromatosis and Schwannomatosis; SH 5 stable hearing; SMILE 5 scaled measurement of improvement in lip excursion; VS 5 vestibular schwannoma; WRS 5 word recognition score. Vestibular schwannomas (VS) are the hallmark of neurofibromatosis 2 (NF2), occurring in .95% of patients. VS develop on the vestibulocochlear nerve and cause significant morbidity due to hearing loss, tinnitus, imbalance, and risk of early mortality from brainstem compression. Hearing loss develops over time in patients with NF2, and these declines are associated with gradual VS growth. 1 Facial weakness is a common sequela of surgery to remove VS and may also occur as a late manifestation of large VS, secondary to facial nerve schwannomas, or as an isolated mononeuropathy. 1 Facial weakness causes articulation difficulties, oral incompetence, lack of ability to smile and express emotions, and inability to blink and protect the cornea, which can lead to vision loss. Facial weakness and hearing loss adversely affect quality of life 2-4 and as such represent important functional outcomes in clinical trials. NATURAL HISTORY OF HEARING LOSS AND FACIAL WEAKNESS IN NF2 The natural history of hearing loss in NF2 has been studied in the prospective multicenter NF2 Natural History Study sponsored by the Department of Defense Consortium. 5 In a cohort of 63 patients (108 VS) within 2 years of the diagnosis of NF2, 27% of the ears experienced a significant loss in pure-tone average, defined as a change of 5 dB compared with baseline. 5 The natural history of facial weakness in NF2 has not been well studied. The best data
Journal of neurosurgery, 2014
Since the 1990 s, Gamma Knife radiosurgery (GKRS) has become the first-line treatment option for small- to medium-size vestibular schwannomas (VSs), especially in patients without mass effect-related symptoms and with functional hearing. The aim of this study was to assess the safety and efficacy of GKRS, in terms of tumor control, hearing preservation, and complications, in a series of 379 consecutive patients treated for VS. Of 523 patients treated at the authors' institution for VS between 2001 and 2010, the authors included 379 who underwent GKRS as the primary treatment. These patients were not affected by Type 2 neurofibromatosis and had clinical follow-up of at least 36 months. Clinical follow-up (mean and median 75.7 and 69.5 months, respectively) was performed for all patients, whereas audiometric and quantitative radiological follow-up examinations were obtained for only 153 and 219 patients, respectively. The patients' ages ranged from 23 to 85 years (mean 59 year...
Prognostic factors of hearing preservation after gamma knife radiosurgery for vestibular schwannoma
Journal of Clinical Neuroscience, 2010
We conducted a prospective study to identify prognostic factors of hearing preservation after gamma knife radiosurgery (GKRS) for vestibular schwannoma (VS). Twenty-seven patients with unilateral VS and serviceable hearing underwent GKRS. The mean lesion diameter was 17.3 mm (range 6.1-30.0 mm), the median marginal dose was 12 Gy (11-15 Gy), and the mean follow-up duration was 35.7 months (9-81 months). The probabilities of hearing preservation after GKRS were calculated using the Kaplan-Meier method. Tumor growth was controlled in 26 of the 27 patients (96.3%), and rates of hearing preservation were 85.1% and 68.1% at 2 and 3 years, respectively. A normal auditory brainstem response (ABR) (p = 0.008) and Gardner-Robertson class I hearing (p = 0.012) before GKRS were found to be significant prognostic factors of a favorable outcome. Our findings suggest that a normal pre GKRS ABR strongly predicts hearing preservation after GKRS. Accordingly, we advise that ABR should be considered with other prognostic factors when GKRS is considered in patients with VS.
Repeat Gamma Knife surgery for vestibular schwannomas
Surgical Neurology International, 2015
Background: Gamma Knife (GK) surgery is a recognized treatment option for the management of small to medium-sized vestibular schwannoma (VS) associated with high-tumor control and low morbidity. When a radiosurgical treatment fails to stop tumor growth, repeat GK surgery can be proposed in selected cases. Methods: A series of 27 GK retreatments was performed in 25 patients with VS; 2 patients underwent three procedures. The median time interval between GK treatments was 45 months. The median margin dose used for the first, second, and third GK treatments was 12 Gy, 12 Gy, and 14 Gy, respectively. Six patients (4 patients for the second irradiation and 2 patients for the third irradiation) with partial tumor regrowth were treated only on the growing part of the tumor using a median margin dose of 13 Gy. The median tumor volume was 0.9, 2.3, and 0.7 cc for the first, second, and third treatments, respectively. Stereotactic positron emission tomography (PET) guidance was used for dose planning in 6 cases. Results: Mean follow-up duration was 46 months (range 24-110). At the last follow-up, 85% of schwannomas were controlled. The tumor volume decreased, remained unchanged, or increased after retreatment in 15, 8, and 4 cases, respectively. Four patients had PET during follow-up, and all showed a significant metabolic decrease of the tumor. Hearing was not preserved after retreatment in any patients. New facial or trigeminal palsy did not occur after retreatment. Conclusions: Our results support the long-term efficacy and low morbidity of repeat GK treatment for selected patients with tumor growth after initial treatment.