Goldbrunner R, Weller M, Regis J, Lund-Johansen M, Stavrinou P, Reuss D, Evans DG, Lefranc F, Sallabanda K, Falini A, Axon P, Sterkers O, Fariselli L, Wick W, Tonn JC.
EANO guideline on the diagnosis and treatment of vestibular schwannoma.
Neuro Oncol. 2020 Jan 11;22(1):31-45. doi: 10.1093/neuonc/noz153.
Abstract/Text
The level of evidence to provide treatment recommendations for vestibular schwannoma is low compared with other intracranial neoplasms. Therefore, the vestibular schwannoma task force of the European Association of Neuro-Oncology assessed the data available in the literature and composed a set of recommendations for health care professionals. The radiological diagnosis of vestibular schwannoma is made by magnetic resonance imaging. Histological verification of the diagnosis is not always required. Current treatment options include observation, surgical resection, fractionated radiotherapy, and radiosurgery. The choice of treatment depends on clinical presentation, tumor size, and expertise of the treating center. In small tumors, observation has to be weighed against radiosurgery, in large tumors surgical decompression is mandatory, potentially followed by fractionated radiotherapy or radiosurgery. Except for bevacizumab in neurofibromatosis type 2, there is no role for pharmacotherapy.
© The Author(s) 2019. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Reznitsky M, Petersen MMBS, West N, Stangerup SE, Cayé-Thomasen P.
The natural history of vestibular schwannoma growth-prospective 40-year data from an unselected national cohort.
Neuro Oncol. 2021 May 5;23(5):827-836. doi: 10.1093/neuonc/noaa230.
Abstract/Text
BACKGROUND: Optimal management of vestibular schwannoma (VS) is still debated and thus international consensus has not been achieved. Treatment options are observation, radiotherapy, and surgery. Knowledge on the natural history of tumor growth is essential for choice of treatment modality. The aim is to present intra-/extrameatal tumor growth and management data from a prospective, unselected national cohort of patients diagnosed with VS during the period 1976-2015.
METHODS: Since 1976, all data from patients diagnosed with sporadic VS in Denmark have been referred to our national treatment center, where they have been entered prospectively into the national database. Data on tumor localization, growth, and treatment were retrieved. Growth definition: >2 mm by linear measurement, in accordance with the Tokyo 2001 consensus-meeting recommendations.
RESULTS: 3637 cases of VS were diagnosed, in which 1304 patients had surgery and 21 received radiotherapy post diagnosis. 2312 patients were observed with mean follow-up of 7.33 years. Of these, 434(19%; 102 intra-and 332 extrameatal tumors) changed to active treatment during the observation period due to tumor growth. 5 years after diagnosis, 21% of the intrameatal tumors exhibited growth during observation, whereas 37% of extrameatal tumors had grown, increasing to 25% intrameatal and 42% extrameatal after 10 years. Following growth, the intrameatal tumors were mostly observed further and the extrameatal mostly underwent surgery. Tumor growth occurred mainly within the first 5 years post diagnosis.
CONCLUSION: This natural history study documents the growth occurrence of both intra-and extrameatal VS during the first 12 years after diagnosis and should be used in patient counseling, management, and treatment decision making.
© The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.
Quesnel AM, McKenna MJ.
Current strategies in management of intracanalicular vestibular schwannoma.
Curr Opin Otolaryngol Head Neck Surg. 2011 Oct;19(5):335-40. doi: 10.1097/MOO.0b013e32834a3fa7.
Abstract/Text
PURPOSE OF REVIEW: The current practitioner is more often managing intracanalicular vestibular schwannomas than in the past, as improved imaging and heightened awareness leads to earlier diagnosis of these tumors. The role of observation, microsurgery, and radiation treatment in the management of intracanalicular tumors continues to evolve. The goal of this article is to evaluate and summarize recent literature pertaining to the management of intracanalicular vestibular schwannomas.
RECENT FINDINGS: Watchful waiting is an important management option for patients with minimal symptoms. The literature on the natural history of small vestibular schwannomas continues to expand, with particular emphasis on the expected hearing outcomes. Microsurgical techniques also focus on hearing preservation. Presence of fundal fluid and good or normal hearing preoperatively are positive predictors of hearing preservation after surgery. Long-term follow-up after radiation therapy for vestibular schwannomas continues to demonstrate excellent tumor control rates, although hearing preservation rates are modest.
SUMMARY: Multiple factors, including status of hearing, presence of vestibular symptoms, patient age, medical comorbidities, institutional outcomes, and patient preferences, help determine the management strategy for patients with an intracanalicular vestibular schwannoma.
Angeli S.
Middle fossa approach: indications, technique, and results.
Otolaryngol Clin North Am. 2012 Apr;45(2):417-38, ix. doi: 10.1016/j.otc.2011.12.010.
Abstract/Text
This article discusses the indications, surgical technique, results, and complications of middle fossa craniotomy (MFC) for vestibular schwannoma surgery, focusing on issues such as serviceable hearing, tumor characteristics, and patient-specific factors that help determine options for therapy. MFC is suitable for intracanalicular vestibular schwannomas that extend less than 1 cm into the cerebellopontine angle in patients with good hearing. With the expanding use of modern imaging, many small tumors are being identified in patients with no or minimal symptoms. Patients with these tumors have three therapy options: (1) stereotactic radiotherapy, (2) microsurgery, and (3) observation (ie, wait-and-scan approach).
Copyright © 2012 Elsevier Inc. All rights reserved.
Arriaga MA, Lin J.
Translabyrinthine approach: indications, techniques, and results.
Otolaryngol Clin North Am. 2012 Apr;45(2):399-415, ix. doi: 10.1016/j.otc.2011.12.009. Epub 2012 Feb 21.
Abstract/Text
This article presents a comprehensive review of the translabyrinthine surgical approach for vestibular schwannoma. Additionally, it addresses the traditional labyrinthectomy and identifies a time-efficient version. Indications and outcomes of the approach are presented, along with detailed procedural technique from opening incision through closure. Complications and management of complications are discussed in detail, as well as postoperative patient care.
Copyright © 2012 Elsevier Inc. All rights reserved.
Elhammady MS, Telischi FF, Morcos JJ.
Retrosigmoid approach: indications, techniques, and results.
Otolaryngol Clin North Am. 2012 Apr;45(2):375-97, ix. doi: 10.1016/j.otc.2012.02.001.
Abstract/Text
This review describes the indications and techniques for the retrosigmoid approach for vestibular schwannoma, as performed by the skull base surgery group at the University of Miami. The authors present background of the retrosigmoid approach, surgical steps, and essential "technical pearls" to address complication avoidance, resulting from their expertise with this surgery.
Copyright © 2012. Published by Elsevier Inc.
Yamakami I, Uchino Y, Kobayashi E, Yamaura A.
Conservative management, gamma-knife radiosurgery, and microsurgery for acoustic neurinomas: a systematic review of outcome and risk of three therapeutic options.
Neurol Res. 2003 Oct;25(7):682-90. doi: 10.1179/016164103101202075.
Abstract/Text
Conservative management, gamma-knife (GK) radiosurgery, and microsurgery are therapeutic options for acoustic neurinomas (ANs). To determine the outcomes and risks of these methods this systematic review analyzed data from 903 patients with conservative management, 1475 with GK radiosurgery, and 5005 with microsurgery from 38 studies identified in MEDLINE searches. Conservative management over a 3.1-year period showed that 51% of ANs showed a tumor growth, an average tumor growth rate was 1.87 mm year-1, 20% of ANs ultimately required surgical intervention, and a third of the patients lost useful hearing. GK radiosurgery significantly reduced the percentage of ANs that enlarged, to 8%, and reduced the percentage that underwent microsurgery to 4.6% over a 3.8-year period. Microsurgery removed 96% of ANs totally, with tumor recurrence, mortality, and major disability rates of 1.8%, 0.63%, and 2.9%, respectively. The majority of ANs grow slowly, but ultimately require intervention. Carrying the risk of hearing loss, conservative management should be supplemented with close follow-up. With a low rate of morbidity, GK radiosurgery suppresses tumor growth and provides good tumor control. Microsurgery provides the best tumor control, although mortality and morbidity are not completely eliminated.
Battista RA.
Gamma knife radiosurgery for vestibular schwannoma.
Otolaryngol Clin North Am. 2009 Aug;42(4):635-54. doi: 10.1016/j.otc.2009.04.009.
Abstract/Text
In 1951, Dr. Lars Leksell from Sweden conceived of what is now known as gamma knife radiosurgery (GKRS). Since Leksell first treated a patient who had a vestibular schwannoma in 1967, there has been a year-to-year increase in the number of patients treated with the gamma knife for vestibular schwannoma. This article outlines the technique of GKRS and discusses the current results of its use to treat vestibular schwannomas. Other topics discussed include tumor control, treatment of recurrent/residual and cystic vestibular schwannomas, and the results of treatment of neurofibromatosis type 2.
Stangerup SE, Thomsen J, Tos M, Cayé-Thomasen P.
Long-term hearing preservation in vestibular schwannoma.
Otol Neurotol. 2010 Feb;31(2):271-5. doi: 10.1097/MAO.0b013e3181c34bda.
Abstract/Text
OBJECTIVE: The aim of the present study was to evaluate the long-term hearing during "wait and scan" management of vestibular schwannomas.
SUBJECTS: During a 33-year period, from 1976 to 2008, 1,144 patients with vestibular schwannoma were allocated to observation by the wait and scan policy, with annual magnetic resonance imaging and audiologic examination. Two complete pure-tone and speech discrimination audiograms were available for 932 patients. In 900 patients (97%), the wait and scan period was at least 1 year. In 377 patients (40%), the observation time was at least 5 years, and in 102 patients (11%), at least 10 years.
RESULTS: At diagnosis, 491 patients (53%) presented with good hearing, that is, speech discrimination better than 70%. After a mean of 4.7 years of observation, 59% of these patients spontaneously preserved good hearing. Of patients with 100% speech discrimination at diagnosis, 69% maintained good hearing after more than 10 years of observation. Of patients with only a small discrimination loss at diagnosis, 38% maintained good hearing.
CONCLUSION: Most vestibular schwannoma patients with 100% speech discrimination at diagnosis maintain good hearing even after many years of observation.
Bin-Alamer O, Abou-Al-Shaar H, Peker S, Samanci Y, Pelcher I, Begley S, Goenka A, Schulder M, Tourigny JN, Mathieu D, Hamel A, Briggs RG, Yu C, Zada G, Giannotta SL, Speckter H, Palque S, Tripathi M, Kumar S, Kaur R, Kumar N, Rogowski B, Shepard MJ, Johnson BA, Trifiletti DM, Warnick RE, Dayawansa S, Mashiach E, Vasconcellos FN, Bernstein K, Schnurman Z, Alzate J, Kondziolka D, Sheehan JP.
Vestibular Schwannoma International Study of Active Surveillance Versus Stereotactic Radiosurgery: The VISAS Study.
Int J Radiat Oncol Biol Phys. 2024 Oct 1;120(2):454-464. doi: 10.1016/j.ijrobp.2024.04.004. Epub 2024 Apr 7.
Abstract/Text
PURPOSE: The present study assesses the safety and efficacy of stereotactic radiosurgery (SRS) versus observation for Koos grade 1 and 2 vestibular schwannoma (VS), benign tumors affecting hearing and neurological function.
METHODS AND MATERIALS: This multicenter study analyzed data from Koos grade 1 and 2 VS patients managed with SRS (SRS group) or observation (observation group). Propensity score matching balanced patient demographics, tumor volume, and audiometry. Outcomes measured were tumor control, serviceable hearing preservation, and neurological outcomes.
RESULTS: In 125 matched patients in each group with a 36-month median follow-up (P = .49), SRS yielded superior 5- and 10-year tumor control rates (99% CI, 97.1%-100%, and 91.9% CI, 79.4%-100%) versus observation (45.8% CI, 36.8%-57.2%, and 22% CI, 13.2%-36.7%; P < .001). Serviceable hearing preservation rates at 5 and 9 years were comparable (SRS 60.4% CI, 49.9%-73%, vs observation 51.4% CI, 41.3%-63.9%, and SRS 27% CI, 14.5%-50.5%, vs observation 30% CI, 17.2%-52.2%; P = .53). SRS were associated with lower odds of tinnitus (OR = 0.39, P = .01), vestibular dysfunction (OR = 0.11, P = .004), and any cranial nerve palsy (OR = 0.36, P = .003), with no change in cranial nerves 5 or 7 (P > .05). Composite endpoints of tumor progression and/or any of the previous outcomes showed significant lower odds associated with SRS compared with observation alone (P < .001).
CONCLUSIONS: SRS management in matched cohorts of Koos grade 1 and 2 VS patients demonstrated superior tumor control, comparable hearing preservation rates, and significantly lower odds of experiencing neurological deficits. These findings delineate the safety and efficacy of SRS in the management of this patient population.
Copyright © 2024 Elsevier Inc. All rights reserved.
Dhayalan D, Tveiten ØV, Finnkirk M, Storstein A, Hufthammer KO, Goplen FK, Lund-Johansen M; V-REX Trial investigators.
Upfront Radiosurgery vs a Wait-and-Scan Approach for Small- or Medium-Sized Vestibular Schwannoma: The V-REX Randomized Clinical Trial.
JAMA. 2023 Aug 1;330(5):421-431. doi: 10.1001/jama.2023.12222.
Abstract/Text
IMPORTANCE: Current guidelines for treating small- to medium-sized vestibular schwannoma recommend either upfront radiosurgery or waiting to treat until tumor growth has been detected radiographically.
OBJECTIVE: To determine whether upfront radiosurgery provides superior tumor volume reduction to a wait-and-scan approach for small- to medium-sized vestibular schwannoma.
DESIGN, SETTING, AND PARTICIPANTS: Randomized clinical trial of 100 patients with a newly diagnosed (<6 months) unilateral vestibular schwannoma and a maximal tumor diameter of less than 2 cm in the cerebellopontine angle as measured on magnetic resonance imaging. Participants were enrolled at the Norwegian National Unit for Vestibular Schwannoma from October 28, 2014, through October 3, 2017; 4-year follow-up ended on October 20, 2021.
INTERVENTIONS: Participants were randomized to receive either upfront radiosurgery (n = 50) or to undergo a wait-and-scan protocol, for which treatment was given only upon radiographically documented tumor growth (n = 50). Participants underwent 5 annual study visits consisting of clinical assessment, radiological examination, audiovestibular tests, and questionnaires.
MAIN OUTCOMES AND MEASURES: The primary outcome was the ratio between tumor volume at the trial end at 4 years and baseline (V4:V0). There were 26 prespecified secondary outcomes, including patient-reported symptoms, clinical examinations, audiovestibular tests, and quality-of-life outcomes. Safety outcomes were the risk of salvage microsurgery and radiation-associated complications.
RESULTS: Of the 100 randomized patients, 98 completed the trial and were included in the primary analysis (mean age, 54 years; 42% female). In the upfront radiosurgery group, 1 participant (2%) received repeated radiosurgery upon tumor growth, 2 (4%) needed salvage microsurgery, and 45 (94%) had no additional treatment. In the wait-and-scan group, 21 patients (42%) received radiosurgery upon tumor growth, 1 (2%) underwent salvage microsurgery, and 28 (56%) remained untreated. For the primary outcome of the ratio of tumor volume at the trial end to baseline, the geometric mean V4:V0 was 0.87 (95% CI, 0.66-1.15) in the upfront radiosurgery group and 1.51 (95% CI, 1.23-1.84) in the wait-and-scan group, showing a significantly greater tumor volume reduction in patients treated with upfront radiosurgery (wait-and-scan to upfront radiosurgery ratio, 1.73; 95% CI, 1.23-2.44; P = .002). Of 26 secondary outcomes, 25 showed no significant difference. No radiation-associated complications were observed.
CONCLUSION AND RELEVANCE: Among patients with newly diagnosed small- and medium-sized vestibular schwannoma, upfront radiosurgery demonstrated a significantly greater tumor volume reduction at 4 years than a wait-and-scan approach with treatment upon tumor growth. These findings may help inform treatment decisions for patients with vestibular schwannoma, and further investigation of long-term clinical outcomes is needed.
TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02249572.
Savardekar AR, Terrell D, Lele SJ, Diaz R, Keesari PR, Trosclair K, Kosty J, Wang CJ, Gardner G, Guthikonda B.
Primary Treatment of Small to Medium (<3 cm) Sporadic Vestibular Schwannomas: A Systematic Review and Meta-Analysis on Hearing Preservation and Tumor Control Rates for Microsurgery versus Radiosurgery.
World Neurosurg. 2022 Apr;160:102-113.e12. doi: 10.1016/j.wneu.2021.11.083. Epub 2021 Nov 24.
Abstract/Text
BACKGROUND: A meta-analysis of patients with sporadic vestibular schwannoma (VS) primarily treated with stereotactic radiosurgery (SRS) or microsurgery (MS) was performed, and hearing preservation outcome (HPO), tumor control (TC), and facial nerve dysfunction (FND) were analyzed.
METHODS: A systematic review was conducted (Medline and Scopus database) for the period January 2010-June 2020 with appropriate MeSH. English language articles for small to medium sporadic VS (<3 cm) using SRS or MS as primary treatment modality, with minimum follow-up of 3 years, were included. Studies had to report an acceptable standardized hearing metric.
RESULTS: Thirty-two studies met the inclusion criteria: 10 MS; 23 radiosurgery, and 1 comparative study included in both. HPO, at approximately 65 months follow-up, were comparable between MS group (10 studies; 809 patients) and SRS group (23 studies; 1234 patients) (56% vs. 59%; P = 0.1527). TC, at approximately 70 months follow-up, was significantly better in the MS group (9 studies; 1635 patients) versus the SRS group (19 studies; 2260 patients) (98% vs. 92%; P < 0.0001). FND, at approximately 12 months follow-up, was significantly higher in the MS group (8 studies; 1101 patients) versus the SRS group (17 studies; 2285 patients) (10% vs. 2%; P < 0.0001).
CONCLUSIONS: MS and SRS are comparable primary treatments for small (<3 cm) sporadic VS with respect to HPO at 5-year follow-up in patients with serviceable hearing at presentation; approximately 50% of patients for both modalities likely lose serviceable hearing by that time point. High TC rates (>90%) were seen with both modalities; MS 98% versus SRS 92%. The posttreatment FND was significantly less with the SRS group (2%) versus the MS group (10%).
Copyright © 2021 Elsevier Inc. All rights reserved.
Whitmeyer M, Brahimaj BC, Beer-Furlan A, Alvi S, Epsten MJ, Crawford F, Byrne RW, Wiet RM.
Resection of vestibular schwannomas after stereotactic radiosurgery: a systematic review.
J Neurosurg. 2021 Sep 1;135(3):881-889. doi: 10.3171/2020.7.JNS2044. Epub 2020 Nov 27.
Abstract/Text
OBJECTIVE: Multiple short series have evaluated the efficacy of salvage microsurgery (MS) after stereotactic radiosurgery (SRS) for treatment of vestibular schwannomas (VSs); however, there is a lack of a large volume of patient data available for interpretation and clinical adaptation. The goal of this study was to provide a comprehensive review of tumor characteristics, management, and surgical outcomes of salvage of MS after SRS for VS.
METHODS: The Medline/PubMed, Scopus, CINAHL, Cochrane Library, and Google Scholar databases were queried according to PRISMA guidelines. All English-language and translated publications were included. Studies lacking adequate study characteristics and outcomes were excluded. Cases involving neurofibromatosis type 2, previous MS, or malignant transformation were excluded when possible.
RESULTS: Twenty studies containing 297 cases met inclusion criteria. Three additional cases from Rush University Medical Center were added for 300 total cases. Tumor growth with or without symptoms was the primary indication for salvage surgery (92.3% of cases), followed by worsening of symptoms without growth (4.6%) and cystic enlargement (3.1%). The average time to MS after SRS was 39.4 months. The average size and volume of tumor at surgery were 2.44 cm and 5.92 cm3, respectively. The surgical approach was retrosigmoid (42.8%) and translabyrinthine (57.2%); 59.5% of patients had a House-Brackmann (HB) grade of I or II. The facial nerve was preserved in 91.5% of cases. Facial nerve preservation and HB grades were lower for the translabyrinthine versus retrosigmoid approach (p = 0.31 and p = 0.18, respectively); however, fewer complications were noted in the translabyrinthine approach (p = 0.29). Gross-total resection (GTR) was completed in 55.7% of surgeries. Studies that predominantly used subtotal resection (STR) were associated with a lower rate of facial nerve injury (5.3% vs 11.3%, p = 0.07) and higher rate of HB grade I or II (72.9% vs 48.0%, p = 0.00003) versus those using predominantly GTR. However, majority STR was associated with a recurrence rate of 3.6% as compared to 1.4% for majority GTR (p = 0.29).
CONCLUSIONS: This study showed that the leading cause of MS after SRS was tumor growth at an average of 39.4 months after radiation. There were no significant differences in outcomes of facial nerve preservation, postoperative HB grade, or complication rate based on surgical approach. Patients who underwent STR showed statistically significant better HB outcomes compared with GTR. MS after SRS was considered by most authors to be more difficult than primary MS. These data support the notion that the surgical goals of salvage surgery are debulking of tumor mass, decreasing compression of the brainstem, and not necessarily pursuing GTR.
