Stereotactic Radiosurgery for Vestibular Schwannomas in Patients with Neurofibromatosis Type 2: An Analysis of Tumor Control, Complications, and Hearing Preservation Rates

2008 ◽  
Vol 2008 ◽  
pp. 17-18 ◽  
Author(s):  
B.J. Balough
Keyword(s):  
Stereotactic Radiosurgery ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Tumor Control

Tumor control and hearing preservation after Gamma Knife radiosurgery for vestibular schwannomas in neurofibromatosis type 2

2010 ◽  
Vol 98 (2) ◽  
pp. 265-270 ◽  
Author(s):  
Manish Singh Sharma ◽  
Rakesh Singh ◽  
Shashank S. Kale ◽  
Deepak Agrawal ◽  
Bhawani Shankar Sharma ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Gamma Knife Radiosurgery ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Tumor Control

STEREOTACTIC RADIOSURGERY FOR VESTIBULAR SCHWANNOMAS IN PATIENTS WITH NEUROFIBROMATOSIS TYPE 2

Neurosurgery ◽  
2007 ◽  
Vol 60 (3) ◽  
pp. 460-470 ◽  
Author(s):  
David Mathieu ◽  
Douglas Kondziolka ◽  
John C. Flickinger ◽  
Ajay Niranjan ◽  
Richard Williamson ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Tumor Volume ◽  
Gamma Knife Radiosurgery ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Factors Affecting

Abstract OBJECTIVE Vestibular schwannomas present significant management challenges in patients with neurofibromatosis Type 2 (NF2). We evaluated the results of gamma knife radiosurgery for the management of these tumors, focusing on tumor response, hearing preservation, and other factors affecting outcomes. METHODS Stereotactic radiosurgery was performed to manage 74 schwannomas in 62 patients. Ipsilateral serviceable hearing was present in 35% of tumors before the procedure. The mean tumor volume was 5.7 cm3. The mean margin and maximum dose used were 14 and 27.5 Gy, respectively. Cox regression analyses were performed to identify factors affecting outcomes. RESULTS The median follow-up period was 53 months, and two patients were lost to follow-up. Actuarial local control rates at were 85, 81, and 81% at 5, 10, and 15 years, respectively. Tumor volume was significant as a predictor of local control. Since 1992, using current radiosurgery techniques (magnetic resonance imaging scan targeting and reduced margin dose to 14 Gy or less), the actuarial serviceable hearing preservation rate is 73% at 1 year, 59% at 2 years, and 48% at 5 years after radiosurgery. Facial neuropathy occurred in 8% of tumors, trigeminal neuropathy occurred in 4%, and vestibular dysfunction occurred in 4%. Radiation dose and tumor volume were predictive of development of new deficits. No radiosurgery-associated secondary tumors or atypical or malignant changes were noted. CONCLUSION Stereotactic radiosurgery is a safe and effective management modality for neurofibromatosis Type 2 vestibular schwannomas. Although results do not seem to be as good as for patients with sporadic unilateral tumors, gamma knife radiosurgery results seem favorable and indicate that radiosurgery should be strongly considered for primary tumor management in selected patients.

scholarly journals NCOG-12. STEREOTACTIC RADIOSURGERY FOR VESTIBULAR SCHWANNOMAS IN NEUROFIBROMATOSIS TYPE 2 PATIENTS: A SYSTEMATIC REVIEW AND META-ANALYSIS

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii131-ii132
Author(s):  
Umberto Tosi ◽  
Omri Maayan ◽  
Anjile An ◽  
Miguel Lavieri ◽  
Sergio Guadix ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Cranial Nerve ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Tumor Control ◽  
Surgical Removal ◽  
Impaired Hearing ◽  
Cranial Nerve Palsies

Abstract BACKGROUND One of the hallmarks of neurofibromatosis type 2 (NF2) is the presence of bilateral vestibular schwannomas (VSs) that result in progressive hearing loss and compression of nearby brainstem structures causing cranial nerve palsies. Treatment of these tumors remains challenging, as both surgical removal and expectant management can result in symptom progression. Stereotactic radiosurgery (SRS) has recently been investigated for the management of NF2-associated VSs; however, the role, promises, and pitfalls of this treatment modality remain unclear. METHODS Ovid MEDLINE, EMBASE, Web of Science, and Cochrane Reviews were searched for studies assessing SRS outcome in NF2-associated VSs only. Primary endpoints included tumor control, serviceable hearing, presence of tinnitus, and cranial nerve V and VII symptoms. RESULTS A total of 16 studies (589 patients harboring 750 tumors) was included in this analysis. Clinical tumor control was achieved in 88% of cases (95% CI: 80%-95%); salvage surgery was needed in 8% (95% CI: 4%-13%) of cases. Treatment resulted in a worsening of pre-treatment serviceable hearing (OR of 0.26, p < 0.01), in an increase of facial nerve impairment (OR of 1.62, p < 0.01), and showed a trend for increase trigeminal nerve impairment as well (OR of 1.42, p = 0.07). The incidence of vestibular symptoms and hydrocephalus requiring shunting could not be assessed since these measures were not reported consistently. CONCLUSIONS The treatment of NF2-associated VS continues to pose a challenge, as current SRS regimens result in impaired hearing and worse cranial nerve comorbidities, despite achieving high tumor control. It remains unclear if these findings have to be intended as treatment complications or, rather, continuous disease progression despite tumor control.

Gamma Knife radiosurgery for treatment of growing vestibular schwannomas in patients with neurofibromatosis Type 2: a matched cohort study with sporadic vestibular schwannomas

2018 ◽  
Vol 128 (1) ◽  
pp. 49-59 ◽  
Author(s):  
Ivo J. Kruyt ◽  
Jeroen B. Verheul ◽  
Patrick E. J. Hanssens ◽  
Henricus P. M. Kunst
Keyword(s):  
Gamma Knife ◽  
Tumor Volume ◽  
Gamma Knife Radiosurgery ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Vestibular Schwannomas ◽  
Tumor Control ◽  
Nerve Function

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. Actuarial tumor control rates were estimated using the Kaplan-Meier method. For patient- and treatment-related factors, a Cox proportional hazards model was used to identify predictors of outcome. Trigeminal, facial, and vestibulocochlear nerve function were assessed before and after treatment. NF2-related VS patients were matched 1:1 with sporadic VS patients who were treated in the same institute, and the same indications for treatment, definitions, and dosimetry were used in order to compare outcomes.RESULTSActuarial tumor control rates in NF2 patients after 1, 3, 5, and 8 years were 98%, 89%, 87%, and 87%, respectively. Phenotype and tumor volume had significant hazard rates of 0.086 and 22.99, respectively, showing that Feiling-Gardner phenotype and a tumor volume not exceeding 6 cm3 both were associated with significantly better outcome. Actuarial rates of serviceable hearing preservation after 1, 3, 5, and 7 years were 95%, 82%, 59%, and 33%, respectively. None of the patients experienced worsening of trigeminal nerve function. Facial nerve function worsened in 1 patient (2.5%). No significant differences in tumor control, hearing preservation, or complications were found in comparing the results of GKRS for NF2-related VS versus GKRS for sporadic VS.CONCLUSIONSWith modern GKRS, the use of low margin doses for treating growing VSs in patients with NF2 demonstrates good long-term tumor control rates. Feiling-Gardner phenotype and tumor volume smaller than 6 cm3 seem to be independently associated with prolonged progression-free survival, highlighting the clinical importance of phenotype assessment before GKRS treatment. In addition, no significant differences in tumor control rates or complications were found in the matched-control cohort analysis comparing GKRS for VS in patients with NF2 and GKRS for sporadic VS. These results show that GKRS is a valid treatment option for NF2-related VS, in addition to being a good option for sporadic VS, particularly in patients with the Feiling-Gardner phenotype and/or tumors that are small to medium in size. Larger tumors in patients with the Wishart phenotype appear to respond poorly to radiosurgery, and other treatment modalities should therefore be considered in such cases.

Multisession Stereotactic Radiosurgery for Vestibular Schwannomas: Single-Institution Experience With 383 Cases

Neurosurgery ◽  
2011 ◽  
Vol 69 (6) ◽  
pp. 1200-1209 ◽  
Author(s):  
Ake Hansasuta ◽  
Clara Y. H. Choi ◽  
Iris C. Gibbs ◽  
Scott G. Soltys ◽  
Victor C. K. Tse ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Tumor Volume ◽  
Medical Center ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Additional Treatment ◽  
Vestibular Schwannomas ◽  
Tumor Control ◽  
Complication Rates ◽  
Kaplan Meier

Abstract BACKGROUND Single-session stereotactic radiosurgery (SRS) treatment of vestibular schwannomas results in excellent tumor control. It is not known whether functional outcomes can be improved by fractionating the treatment over multiple sessions. OBJECTIVE To examine tumor control and complication rates after multisession SRS. METHODS Three hundred eighty-three patients treated with SRS from 1999 to 2007 at Stanford University Medical Center were retrospectively reviewed. Ninety percent were treated with 18 Gy in 3 sessions, targeting a median tumor volume of 1.1 cm3 (range, 0.02-19.8 cm3). RESULTS During a median follow-up duration of 3.6 years (range, 1-10 years), 10 tumors required additional treatment, resulting in 3- and 5-year Kaplan-Meier tumor control rates of 99% and 96%, respectively. Five-year tumor control rate was 98% for tumors > 3.4 cm3. Neurofibromatosis type 2–associated tumors were associated with worse tumor control (P = .02). Of the 200 evaluable patients with pre-SRS serviceable hearing (Gardner-Robertson grade 1 and 2), the crude rate of serviceable hearing preservation was 76%. Smaller tumor volume was associated with hearing preservation (P = .001). There was no case of post-SRS facial weakness. Eight patients (2%) developed trigeminal dysfunction, half of which was transient. CONCLUSION Multisession SRS treatment of vestibular schwannomas results in an excellent rate of tumor control. The hearing, trigeminal nerve, and facial nerve function preservation rates reported here are promising.

scholarly journals Long-term follow-up studies of Gamma Knife surgery for patients with neurofibromatosis Type 2

2014 ◽  
Vol 121 (Suppl_2) ◽  
pp. 143-149 ◽  
Author(s):  
Shibin Sun ◽  
Ali Liu
Keyword(s):  
Gamma Knife ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Vestibular Schwannomas ◽  
Tumor Control ◽  
History Of ◽  
The Mean

ObjectThe aim of this study was to evaluate long-term clinical outcomes after Gamma Knife surgery (GKS) for patients with neurofibromatosis Type 2 (NF2) and the role of GKS in the management of NF2.MethodsFrom December 1994 through December 2008, a total of 46 patients (21 male, 25 female) with NF2 underwent GKS and follow-up evaluation for at least 5 years at the Gamma Knife Center of the Beijing Neurosurgical Institute. GKS was performed using the Leksell Gamma Knife Models B and C. The mean age of the patients was 30 years (range 13–59 years). A family history of NF2 was found for 9 (20%) patients. The NF2 phenotype was thought to be Wishart for 20 (44%) and Feiling-Gardner for 26 (56%) patients. Among these 46 patients, GKS was performed to treat 195 tumors (73 vestibular schwannomas and 122 other tumors including other schwannomas and meningiomas). For vestibular schwannomas, the mean volume was 5.1 cm3 (median 3.6 cm3, range 0.3–27.3 cm3), the mean margin dose was 12.9 Gy (range 10–14 Gy), and the mean maximum dose was 27.3 Gy (range 16.2–40 Gy). For other tumors, the mean volume was 1.7 cm3 (range 0.3–5.5 cm3), the mean margin dose was 13.3 Gy (range 11–14 Gy), and the mean maximum dose was 26.0 Gy (range 18.0–30.4 Gy). The median duration of follow-up was 109 months (range 8–195 months).ResultsFor the 73 vestibular schwannomas that underwent GKS, the latest follow-up MR images demonstrated regression of 30 (41%) tumors, stable size for 31 (43%) tumors, and enlargement of 12 (16%) tumors. The total rate of tumor control for bilateral vestibular schwannomas in patients with NF2 was 84%. Of the 122 other types of tumors that underwent GKS, 103 (85%) showed no tumor enlargement. The rate of serviceable hearing preservation after GKS was 31.9% (15/47). The actuarial rates for hearing preservation at 3 years, 5 years, 10 years, and 15 years were 98%, 93%, 44%, and 17%, respectively. Of the 46 patients, 22 (48%) became completely bilaterally deaf, 17 (37%) retained unilateral serviceable hearing, and 7 (15%) retained bilateral serviceable hearing. The mean history of the disease course was 12 years (range 5–38 years).ConclusionsGKS was confirmed to provide long-term local tumor control for small- to medium-sized vestibular schwannomas and other types of tumors, although vestibular schwannomas in patients with NF2 responded less well than did unilateral sporadic vestibular schwannomas. Phenotype is the most strongly predictive factor of final outcome after GKS for patients with NF2. The risk for loss of hearing is high, whereas the risk for other cranial nerve complications is low.

scholarly journals Minimal Effect of Bevacizumab Treatment on Residual Vestibular Schwannomas after Partial Resection in Young Neurofibromatosis Type 2 Patients

Cancers ◽  
2019 ◽  
Vol 11 (12) ◽  
pp. 1862 ◽  
Author(s):  
Isabel Gugel ◽  
Lan Kluwe ◽  
Julian Zipfel ◽  
Christian Teuber ◽  
Marcos Tatagiba ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Tumor Volume ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Partial Resection ◽  
Vestibular Schwannomas ◽  
Minimal Growth ◽  
Bevacizumab Treatment

Hearing-preserving partial resection of neurofibromatosis type 2 (NF2) associated vestibular schwannomas (VS) is a preferred treatment strategy, particularly for children and adolescents. However, the residual tumors do grow and lead at some point to continued hearing deterioration. An adjuvant bevacizumab treatment may provide an option for slowing down this process. In this retrospective study, we reviewed tumor volume and hearing data of 16 operated VS in nine patients younger than 30 years over a period of 63 to 142 months. All these patients had one or more bevacizumab treatment periods and most of them had a non-treatment period after surgery. Four different patterns of growth were observed for the residual tumors: (1) growth in the non-treatment periods, which slowed down in the treatment periods; (2) growth slowed down in one but not in another on-period; (3) unaffected growth; (4) no or minimal growth regardless of the treatment. Neither radiological regression of tumor volume nor hearing improvement were observed in the treatment periods. Accelerated hearing deterioration was observed in several non-treatment periods, but also in some treatment periods. No straightforward correlation can be drawn between tumor growth and hearing scores. Tumor growth and worsening of hearing between two measurement points were slightly less in the treatment periods; however, the differences were not significant, because variations were large. In conclusion, our comprehensive follow-up on 16 VS in nine NF2 patients did show heterogenous effects of bevacizumab on small residual vestibular schwannomas after surgery both regarding tumor size and hearing preservation. Thus, smaller and slower growing tumor residuals seem to behave differently to bevacizumab than reported for not-operated faster growing VS.

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