Facial Nerve Outcomes After Vestibular Schwannoma Microsurgical Resection in Neurofibromatosis Type 2

2020 ◽  
pp. 019459982095414
Author(s):  
Catherine Sobieski ◽  
Daniel E. Killeen ◽  
Samuel L. Barnett ◽  
Bruce E. Mickey ◽  
Jacob B. Hunter ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Vestibular Schwannoma ◽  
Tumor Volume ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Diameter ◽  
Microsurgical Resection

Objective The aim of this study is to investigate facial nerve outcomes after microsurgical resection in neurofibromatosis type 2 (NF2) compared to sporadic tumors. Study Design Single institutional retrospective chart review. Setting Tertiary referral center. Methods All adult patients with NF2 vestibular schwannoma (VS) or sporadic VS who underwent microsurgical resection from 2008 to 2019 with preoperative magnetic resonance imaging (MRI) and 1 year of postsurgical follow-up were included. The primary outcome measure was postoperative House-Brackmann (HB) facial nerve score measured at first postoperative visit and after at least 10 months. Results In total, 161 sporadic VSs and 14 NF2 VSs met inclusion criteria. Both median tumor diameter (NF2, 33.5 mm vs sporadic, 24 mm, P = .0011) and median tumor volume (NF2, 12.4 cm3 vs sporadic, 2.9 cm3, P = .0005) were significantly greater in patients with NF2. The median follow-up was 24.9 months (range, 12-130.1). Median facial nerve function after 1 year for patients with NF2 was HB 3 (range, 1-6) compared to HB 1 (range, 1-6) for sporadic VS ( P = .001). With multivariate logistic regression, NF2 tumors (odds ratio [OR] = 13.9, P = .001) and tumor volume ≥3 cm3 (OR = 3.6, P = .025) were significantly associated with HB ≥3 when controlling for age, sex, extent of tumor resection, translabyrinthine approach, and prior radiation. Conclusion Tumor volume >3 cm3 and NF2 tumors are associated with poorer facial nerve outcomes 1 year following microsurgical resection.

Sporadic unilateral vestibular schwannoma in the pediatric population

2009 ◽  
Vol 4 (2) ◽  
pp. 125-129 ◽  
Author(s):  
Brian P. Walcott ◽  
Ganesh Sivarajan ◽  
Bronislava Bashinskaya ◽  
Douglas E. Anderson ◽  
John P. Leonetti ◽  
...  
Keyword(s):  
Facial Nerve ◽  
Tumor Size ◽  
Pediatric Population ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Function ◽  
Facial Nerve Function ◽  
Duration Of Symptoms

Object Vestibular schwannomas (VSs) are rare in the pediatric population. Most often, these lesions manifest as a bilateral disease process in the setting of neurofibromatosis Type 2. Even in the absence of additional clinical diagnostic criteria, the presentation of a unilateral VS in a young patient may be a harbinger of future penetrance for this hereditary tumor syndrome. Methods The authors retrospectively reviewed the charts of a cohort of 7 patients who presented with apparently sporadic, unilateral VSs. These patients had previously undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were reviewed with emphasis on facial nerve function and follow-up for signs and symptoms of a heritable disorder. Results All patients underwent microsurgical resection in a multidisciplinary effort by the senior authors. The average tumor size was 4.57 cm, with an average duration of symptoms prior to definitive diagnosis of 31.2 months. The tumor size at the time of presentation followed a trend different from reports in adults, while the duration of symptoms did not. At a follow-up average of 6.3 years (range 1–12 years), 100% of patients demonstrated good facial function (House-Brackmann Grade I or II). No patient in this cohort demonstrated symptoms, objective signs, or genetic analysis indicating the presence of neurofibromatosis Type 2. Conclusions Diagnosis and management of sporadic, unilateral VSs in children is complicated by clinical presentations and surgical challenges unique from their adult counterparts. Careful consideration should be given to a heritable genetic basis for sporadic unilateral VS in the pediatric population. Results of genetic testing do not preclude the necessity for long-term follow-up and systemic investigation. In patients who present with large tumors, preliminary experience leads the authors to suggest that a combined retrosigmoid-translabyrinthine approach offers the greatest opportunity for preservation of facial nerve function.

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.

Conservative Management of Bilateral Vestibular Schwannomas in Neurofibromatosis Type 2 Patients: Hearing and Tumor Growth Results

Neurosurgery ◽  
2013 ◽  
Vol 72 (6) ◽  
pp. 907-914 ◽  
Author(s):  
Matthieu Peyre ◽  
Stéphane Goutagny ◽  
Alpha Bah ◽  
Daniele Bernardeschi ◽  
Béatrice Larroque ◽  
...  
Keyword(s):  
Growth Rate ◽  
Tumor Growth ◽  
Conservative Management ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Growth Rate ◽  
Tumor Diameter ◽  
The Mean

Abstract BACKGROUND: As new treatment modalities develop for the management of vestibular schwannomas (VS) in patients with neurofibromatosis type 2, it remains crucial to ascertain the natural history of the disease. OBJECTIVE: To determine the relationship between hearing and tumor growth in patients undergoing conservative VS management. METHODS: Patients harboring bilateral VS with at least 1 year of radiological follow-up were selected. Conservative management was proposed based on the small tumor size and/or serviceable hearing at presentation. Tumor size was calculated by using the 2-component box model and reported as mean tumor diameter. Hearing was evaluated by using pure-tone average and the American Academy of Otololaryngologists and Head and Neck Surgery classification. RESULTS: Forty-six patients harboring 92 VS were included. The mean clinical and radiological follow-up times were 6.0 and 4.2 years, respectively. The mean tumor diameter was 13 mm at presentation and 20 mm at the end of follow-up. Mean tumor growth rate was 1.8 mm/year. During follow-up, 17 patients (37%) underwent surgery for VS. Surgery-free rate for VS was 88% at 5 years. The number of patients with at least 1 serviceable ear was 39 (85%) at presentation and 34 (74%) at the end of follow-up, including 22 (66%) with binaural serviceable hearing maintained. There was no statistical correlation between tumor growth rate and preservation of serviceable hearing. Tumor growth rates and age at presentation were inversely correlated. CONCLUSION: This study illustrates the high variability among neurofibromatosis type 2 patients regarding hearing status and VS growth rate and justifies the choice of initial conservative management in selected cases.

scholarly journals Malignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient

Neurosurgery ◽  
2017 ◽  
Vol 83 (1) ◽  
pp. 38-42 ◽  
Author(s):  
Andrew T King ◽  
Scott A Rutherford ◽  
Charlotte Hammerbeck-Ward ◽  
Simon K Lloyd ◽  
Simon R Freeman ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Increased Risk

Abstract BACKGROUND The published literature suggests that malignant peripheral nerve sheath tumors (MPNST) occur at increased frequency in neurofibromatosis type 2 (NF2). A recent review based on incidence data in North America showed that 1 per 1000 cerebellopontine angle nerve sheath tumors were malignant. OBJECTIVE To determine whether MPNST occurred spontaneously in NF2 by reviewing our NF2 database. METHODS The prospective database consists of 1253 patients with NF2. One thousand and nine are known to be alive at last follow-up. The presence and laterality/pathology of vestibular schwannoma at diagnosis and last follow-up was sought. RESULTS There were no cases of spontaneous MPNST with 2114 proven (n = 1150) and presumed benign (n = 964) vestibular schwannomas found. Two patients had developed MPNST (1 presumed) after having previously undergone stereotactic radiosurgery for a vestibular schwannoma. CONCLUSION In this series, and from the literature, malignant transformation of a vestibular schwannoma was not a feature of NF2 in the unirradiated patient. NF2 patients should not be told that they have an increased risk of malignant change in a vestibular schwannoma unless they undergo radiation treatment. However, very much larger datasets are required before it can be determined whether there is any association between NF2 and MPNST in the unirradiated patient.

Skull Base Meningiomas in Patients with Neurofibromatosis Type 2: An International Multicenter Study Evaluating Stereotactic Radiosurgery

2021 ◽  
Author(s):  
Henry Ruiz-Garcia ◽  
Daniel M. Trifiletti ◽  
Nasser Mohammed ◽  
Yi-Chieh Hung ◽  
Zhiyuan Xu ◽  
...  
Keyword(s):  
Skull Base ◽  
Tumor Volume ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Control ◽  
Invasive Approach ◽  
Skull Base Meningiomas ◽  
International Multicenter

Abstract Objective Meningiomas are the second most common tumors in neurofibromatosis type 2 (NF-2). Microsurgery is challenging in NF-2 patients presenting with skull base meningiomas due to the intrinsic risks and need for multiple interventions over time. We analyzed treatment outcomes and complications after primary Gamma Knife radiosurgery (GKRS) to delineate its role in the management of these tumors. Methods An international multicenter retrospective study approved by the International Radiosurgery Research Foundation was performed. NF-2 patients with at least one growing and/or symptomatic skull base meningioma and 6-month follow-up after primary GKRS were included. Clinical and radiosurgical parameters were recorded for analysis. Results In total, 22 NF-2 patients with 54 skull base meningiomas receiving GKRS as primary treatment met inclusion criteria. Median age at GKRS was 38 years (10–79 years). Most lesions were located in the posterior fossa (55.6%). Actuarial progression free survival (PFS) rates were 98.1% at 2 years and 90.0% at 5 and 10 years. The median follow-up time after initial GKRS was 5.0 years (0.6–25.5 years). Tumor volume at GKRS was a predictor of tumor control. Lesions >5.5 cc presented higher chances to progress after radiosurgery (p = 0.043). Three patients (13.64%) developed adverse radiation effects. No malignant transformation or death due to meningioma or radiosurgery was reported. Conclusions GKRS is effective and safe in the management of skull base meningiomas in NF-2 patients. Tumor volume deserve greater relevance during clinical decision-making regarding the most appropriate time to treat. GKRS offers a minimally invasive approach of particular interest in this specific group of patients.

scholarly journals Disease course of Neurofibromatosis Type 2; a 30-year follow-up study of 353 patients seen at a single institution

2020 ◽  
Author(s):  
Claire Forde ◽  
Andrew T King ◽  
Scott A Rutherford ◽  
Charlotte Hammerbeck-Ward ◽  
Simon K Lloyd ◽  
...  
Keyword(s):  
De Novo ◽  
Radiation Treatment ◽  
Univariate Analysis ◽  
Age At Onset ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Disease Course ◽  
Number Of Patients

Abstract Background Limited data exists on the disease course of Neurofibromatosis Type 2 (NF2) to guide clinical trial design. Methods A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990–2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred and inheritance type. Interventions for NF2-related tumours were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death. Results Three-hundred-and-fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65 respectively per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting <16 and >40 years had poorer overall survival than those presenting at 26-39 years (P=0.03 and P=0.02 respectively) but those presenting between 16-39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P=0.004). Conclusion Understanding disease course improves prognostication, allowing for better informed decisions about care.

scholarly journals Contextualizing the Modern Epidemiology of Neurofibromatosis Type 2 in an Era of Heightened Detection of Sporadic Vestibular Schwannoma

2020 ◽  
Vol 41 (4) ◽  
pp. e501-e506 ◽  
Author(s):  
Eric M. Dowling ◽  
John P. Marinelli ◽  
Christine M. Lohse ◽  
Matthew L. Carlson
Keyword(s):  
Vestibular Schwannoma ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2

Resection of 2 Intradural Extramedullary Cervical Spine Tumors in a Patient With Neurofibromatosis Type 2: 3-Dimensional Operative Video

2018 ◽  
Vol 16 (2) ◽  
pp. 274-274
Author(s):  
Simone E Dekker ◽  
Chad A Glenn ◽  
Thomas A Ostergard ◽  
Osmond C Wu ◽  
Fernando Alonso ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Spine ◽  
Treatment Options ◽  
Tumor Resection ◽  
Neurofibromatosis Type ◽  
Cord Compression ◽  
Neurofibromatosis Type 2 ◽  
3 Dimensional ◽  
Intradural Extramedullary

Abstract This 3-dimensional operative video illustrates resection of 2 cervical spine schwannomas in a 19-yr-old female with neurofibromatosis type 2. The patient presented with lower extremity hyperreflexity and hypertonicity. Magnetic resonance imaging (MRI) demonstrated 2 contrast-enhancing intradural extramedullary cervical spine lesions causing spinal cord compression at C4 and C5. The patient underwent a posterior cervical laminoplasty with a midline dural opening for tumor resection. Curvilinear spine cord compression is demonstrated in the operative video. After meticulous dissection, the tumors were resected without complication. The dural closure was performed in watertight fashion followed by laminoplasty using osteoplastic titanium miniplates and screws. Postoperative MRI demonstrated gross total resection with excellent decompression of the spinal cord. The postoperative course was uneventful. The natural history of this disease, treatment options, and potential complications are discussed.

Bevacizumab for Hearing Preservation in Neurofibromatosis Type 2: Emphasis on Patient-Reported Outcomes and Toxicities

2018 ◽  
Vol 160 (3) ◽  
pp. 526-532 ◽  
Author(s):  
Pavlina Sverak ◽  
Meredith E. Adams ◽  
Stephen J. Haines ◽  
Samuel C. Levine ◽  
David Nascene ◽  
...  
Keyword(s):  
Patient Reported Outcomes ◽  
Tumor Volume ◽  
Neurofibromatosis Type ◽  
Hearing Preservation ◽  
Neurofibromatosis Type 2 ◽  
Hearing Improvement ◽  
Radiographic Response ◽  
Bevacizumab Treatment ◽  
Patient Reported

Objective Bevacizumab for hearing preservation in patients with neurofibromatosis type 2 (NF2) is an emerging practice. We set out to characterize the effectiveness and toxicity of bevacizumab in our patient group. Study Design Case series with chart review. Setting Tertiary referral center. Subjects and Methods Seventeen consecutive patients with NF2 received bevacizumab treatment for vestibular schwannomas, including 2 patients treated to maintain cochlear implant performance. Volumetric analysis of serial magnetic resonance imaging scans was used to evaluate radiographic response, and hearing response was evaluated with serial audiograms. Patient-reported outcomes were also assessed, including subjective hearing improvement, changes in tinnitus, vertigo, headaches, ear pain, and improvement in ability to communicate via telephone. Results A positive radiographic response occurred in 8 of 17 (47%) patients and the median tumor volume change was a tumor decrease of 19%. A positive hearing response was recorded in 5 of 9 (56%) patients. Two patients had a word recognition score improvement over 40%. There was an approximately 40% improvement in patient-reported outcomes. Primary toxicities included hypertension, proteinuria, dysgeusia, and amenorrhea. Conclusion Bevacizumab treatment was followed by hearing improvement in 56% of patients, while decreased tumor volume was noted in 47%. These outcomes agree favorably with prior reported series. There were significant improvements in patient-reported outcomes that have not been described previously.

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