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.

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.

Conservative Treatment of Patients with Acoustic Tumors

Neurosurgery ◽  
1991 ◽  
Vol 28 (5) ◽  
pp. 646-651 ◽  
Author(s):  
Joshua B. Bederson ◽  
Klaus von Ammon ◽  
Werner W. Wichmann ◽  
Gazi M. Yasargil
Keyword(s):  
Growth Rate ◽  
Conservative Treatment ◽  
Tumor Growth ◽  
Tumor Size ◽  
Tumor Growth Rate ◽  
Computed Tomographic ◽  
High Incidence ◽  
The Mean ◽  
Slow Growing

Abstract Seventy of 178 patients with acoustic tumors initially were treated conservatively and have been followed up for an average of 26 ± 2 months. The tumor size was determined by the mean maximum anteroposterior and mediolateral diameters, using computed tomographic or magnetic resonance imaging scans obtained sequentially throughout the follow-up period. The average tumor growth was 1.6 ± 0.4 mm the 1st year, and 1.9 ± 1.0 mm the 2nd year (range, -2 to 17 mm/y): 4 tumors showed apparent regression, 28 (40%) had no detectable growth, and 37 (53%) exhibited growth (average, 3.8 ± 1.2 mm/y). Within individual patients, the tumor growth rate determined during the 1st year of follow-up was predictive of tumor growth rate during the following year. Rapid tumor growth or clinical deterioration in 9 of the 70 patients (13%) who initially were treated conservatively necessitated subsequent surgery an average of 14 ± 5 months after the patient was initially seen. This group had a larger initial tumor size (27.0 ± 3.4 mm vs. 21.3 ± 0.9 mm, P<0.05), and a faster 1-year growth rate (7.9 ± 2.3 mm/y vs. 1.3 ± 0.3 mm/y, P<0.05) than the 61 patients who did not require surgery. Two patients, however, experienced neurological deterioration that required surgery, even though there was no tumor growth. The high incidence of acoustic tumors with no detectable growth or apparent spontaneous regression must be taken into account when evaluating the indications for surgery and the efficacy of radiotherapy. Beacuse surgery carries some risk and acoustic tumors are generally slow growing, a trial of conservative treatment is possible in selected patients, provided serial radiological studies are obtained. Knowledge of the tumor growth rate established by these studies may be helpful in the treatment of individual patients.

Conservative Management or Gamma Knife Radiosurgery for Vestibular Schwannoma

Neurosurgery ◽  
2013 ◽  
Vol 73 (1) ◽  
pp. 48-57 ◽  
Author(s):  
Cathrine Nansdal Breivik ◽  
Roy Miodini Nilsen ◽  
Erling Myrseth ◽  
Paal Henning Pedersen ◽  
Jobin K. Varughese ◽  
...  
Keyword(s):  
Growth Rate ◽  
Tumor Growth ◽  
Gamma Knife ◽  
Conservative Management ◽  
Regression Models ◽  
Gamma Knife Radiosurgery ◽  
Rank Test ◽  
Tumor Growth Rate ◽  
Log Rank Test

Abstract BACKGROUND: There are few reports about the course of vestibular schwannoma (VS) patients following gamma knife radiosurgery (GKRS) compared with the course following conservative management (CM). In this study, we present prospectively collected data of 237 patients with unilateral VS extending outside the internal acoustic canal who received either GKRS (113) or CM (124). OBJECTIVE: The aim was to measure the effect of GKRS compared with the natural course on tumor growth rate and hearing loss. Secondary end points were postinclusion additional treatment, quality of life (QoL), and symptom development. METHODS: The patients underwent magnetic resonance imaging scans, clinical examination, and QoL assessment by SF-36 questionnaire. Statistics were performed by using Spearman correlation coefficient, Kaplan-Meier plot, Poisson regression model, mixed linear regression models, and mixed logistic regression models. RESULTS: Mean follow-up time was 55.0 months (26.1 standard deviation, range 10-132). Thirteen patients were lost to follow-up. Serviceable hearing was lost in 54 of 71 (76%) (CM) and 34 of 53 (64%) (GKRS) patients during the study period (not significant, log-rank test). There was a significant reduction in tumor volume over time in the GKRS group. The need for treatment following initial GKRS or CM differed at highly significant levels (log-rank test, P < .001). Symptom and QoL development did not differ significantly between the groups. CONCLUSION: In VS patients, GKRS reduces the tumor growth rate and thereby the incidence rate of new treatment about tenfold. Hearing is lost at similar rates in both groups. Symptoms and QoL seem not to be significantly affected by GKRS.

Conservative management of 386 cases of unilateral vestibular schwannoma: tumor growth and consequences for treatment

2009 ◽  
Vol 110 (4) ◽  
pp. 662-669 ◽  
Author(s):  
Wissame El Bakkouri ◽  
Romain E. Kania ◽  
Jean-Pierre Guichard ◽  
Guillaume Lot ◽  
Philippe Herman ◽  
...  
Keyword(s):  
Growth Rate ◽  
Mr Imaging ◽  
Tumor Growth ◽  
Conservative Management ◽  
Tumor Size ◽  
Imaging Study ◽  
Tumor Growth Rate ◽  
Slow Growth Rate ◽  
Delay In Diagnosis

Object The object of this study was to evaluate the natural history, pattern, and occurrence of tumor growth and its consequences for treatment of small-sized vestibular schwannomas (VSs). Methods From 1990 to 2005, 386 patients underwent conservative management for VS because of the following: age > 60 years, poor health/medical risks, risk of deterioration of good hearing, small tumor size, minimal or no incapacitating symptoms, and/or patient preference. Tumor size was measured by MR imaging according to the guidelines of the Committee on Hearing and Equilibrium. The first MR imaging study was performed 1 year after diagnosis, and subsequent imaging was performed yearly or every 2 years depending on the appearance of new symptoms, tumor growth, or both. Results Sixty-one patients were lost to follow-up the first year after presentation. Of the 325 patients for whom 1-year follow-up data were available, 39 showed tumor growth ≥ 3 mm. Conservative management was discontinued for these 39 patients. The patients who returned for follow-up were evaluated at 1- or 2-year intervals depending on tumor growth. The authors extrapolated to obtain data for 2-year intervals, yielding data for 160, 56, 21, and 8 patients at 3, 5, 7, and 9 years after initial presentation, respectively. The overall mean tumor growth rate (±standard deviation) was 1.15 ± 2.4 mm/year. This rate was estimated by pooling all values of tumor growth that had been determined for all patients and dividing by the total number of “events,” with each assessment constituting an event. In 58.6% of patients, the annual tumor growth rate was < 1 mm/year; in 29.2%, 1–3 mm/year; and in 12.2%, ≥ 3 mm/ year. The growth rates of intrameatal (1.02 ± 1.8 mm/year) and extrameatal (1.40 ± 3.1 mm/year) tumors did not differ significantly. No significant association was found between tumor growth rate and sex, age, initial hearing status, or initial tumor grade. Delay in diagnosis was the only significant factor associated with tumor growth rate. During follow-up, conservative management was discontinued for 77 (23.7%) of the 325 patients for whom at least 12-month follow-up data were available; surgery was performed in 60 (77.9%) and radiation therapy in 17 (22.1%). Conclusions The results of this study support the role of a conservative “wait-and-scan” policy of management for small-sized VSs because most have a slow growth rate. Long-term neuroimaging follow-up is needed even with non-growing tumors.

scholarly journals Long-term natural history of neurofibromatosis Type 2–associated intracranial tumors

2012 ◽  
Vol 117 (1) ◽  
pp. 109-117 ◽  
Author(s):  
Michael S. Dirks ◽  
John A. Butman ◽  
H. Jeffrey Kim ◽  
Tianxia Wu ◽  
Keaton Morgan ◽  
...  
Keyword(s):  
Growth Rate ◽  
Natural History ◽  
Growth Pattern ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Intracranial Tumors ◽  
History Of

Object Neurofibromatosis Type 2 (NF2) is a heritable tumor predisposition syndrome that leads to the development of multiple intracranial tumors, including meningiomas and schwannomas. Because the natural history of these tumors has not been determined, their optimal management has not been established. To define the natural history of NF2-associated intracranial tumors and to optimize management strategies, the authors evaluated long-term clinical and radiographic data in patients with NF2. Methods Consecutive NF2 patients with a minimum of 4 years of serial clinical and MRI follow-up were analyzed. Results Seventeen patients, 9 males and 8 females, were included in this analysis (mean follow-up 9.5 ± 4.8 years, range 4.0–20.7 years). The mean age at initial evaluation was 33.2 ± 15.5 years (range 12.3–57.6 years). Patients harbored 182 intracranial neoplasms, 164 of which were assessable for growth rate analysis (18 vestibular schwannomas [VSs], 11 nonvestibular cranial nerve [CN] schwannomas, and 135 meningiomas) and 152 of which were assessable for growth pattern analysis (15 VSs, 9 nonvestibular CN schwannomas, and 128 meningiomas). New tumors developed in patients over the course of the imaging follow-up: 66 meningiomas, 2 VSs, and 2 nonvestibular CN schwannomas. Overall, 45 tumors (29.6%) exhibited linear growth, 17 tumors (11.2%) exhibited exponential growth, and 90 tumors (59.2%) displayed a saltatory growth pattern characterized by alternating periods of growth and quiescence (mean quiescent period 2.3 ± 2.1 years, range 0.4–11.7 years). Further, the saltatory pattern was the most frequently identified growth pattern for each tumor type: meningiomas 60.9%, VSs 46.7%, and nonvestibular schwannoma 55.6%. A younger age at the onset of NF2-related symptoms (p = 0.01) and female sex (p = 0.05) were associated with an increased growth rate in meningiomas. The identification of saltatory growth in meningiomas increased with the duration of follow-up (p = 0.01). Conclusions Neurofibromatosis Type 2–associated intracranial tumors most frequently demonstrated a saltatory growth pattern. Because new tumors can develop in NF2 patients over their lifetime and because radiographic progression and symptom formation are unpredictable, resection may be best reserved for symptom-producing tumors. Moreover, establishing the efficacy of nonsurgical therapeutic interventions must be based on long-term follow-up (several years).

scholarly journals Stereotactic radiosurgery in the management of acoustic neuromas associated with neurofibromatosis Type 2

1999 ◽  
Vol 6 (3) ◽  
pp. E1
Author(s):  
Brian R. Subach ◽  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
David J. Bissonette ◽  
John C. Flickinger ◽  
...  
Keyword(s):  
Stereotactic Radiosurgery ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Tumor Control ◽  
Nerve Function ◽  
Tumor Control Rate ◽  
Acoustic Neuromas ◽  
The Mean

Object Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To better define the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions. Methods Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml and the mean tumor margin dose was 15 Gy (range 12–20 Gy). The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 (36%) tumors regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner-Robertson Class I or II) was preserved in six (43%) of 14 patients and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House-Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients. Conclusions Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.

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 &lt;16 and &gt;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.

Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2

2003 ◽  
Vol 99 (3) ◽  
pp. 480-483 ◽  
Author(s):  
Goro Otsuka ◽  
Kiyoshi Saito ◽  
Tetsuya Nagatani ◽  
Jun Yoshida
Keyword(s):  
Symptom Onset ◽  
Survival Rates ◽  
Neurofibromatosis Type ◽  
Neurofibromatosis Type 2 ◽  
Vestibular Schwannomas ◽  
Content Type ◽  
Fine Print

Object. Neurofibromatosis Type 2 (NF2) is an intractable disorder predisposing to multiple, recurrent tumors of the central nervous system (CNS). To clarify the survival rate and characteristics that predict poor survival, we retrospectively reviewed clinical data in cases of NF2. Methods. From among 283 patients with neurofibromatosis who had been registered in a nationwide study in Japan between 1986 and 1987, 74 patients with bilateral vestibular schwannomas were analyzed. The mean duration of follow up after diagnosis was 121 months (range 2–287 months). Results of a Kaplan—Meier product-limit analysis indicated that overall 5-, 10-, and 20-year patient survival rates following diagnosis of NF2 were 85, 67, and 38%, respectively. Early onset of the initial symptom significantly compromised survival; 5-, 10-, and 20-year survival rates in patients with symptom onset at an age younger than 25 years were 80, 60, and 28%, respectively, whereas in patients with symptom onset at an age of 25 years or older the rates were 100, 87, and 62%, respectively. Patients with small vestibular schwannomas at diagnosis (< 2 cm in diameter) had better rates of survival. Other variables such as sex, additional tumors in the CNS, or dermal abnormalities did not significantly affect survival. Conclusions. This first report of long-term follow-up results concerning the survival of patients with NF2 indicates an adverse effect of early symptom onset.

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.

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