Cystic Vestibular Schwannomas Research Articles

The Dilemma of Early Postoperative Magnetic Resonance Imaging

Postoperative magnetic resonance imaging (MRI) is critical to the clinical decision-making process for patients undergoing resection of intracranial tumors. The accuracy of immediate postoperative MRI in determining the presence of residual disease following intracranial tumor resection, however, has not been studied. A 57-year-old man underwent an uncomplicated retrosigmoid craniotomy for the resection of a cystic vestibular schwannoma. Immediate gadolinium-enhanced postoperative MRI, performed within 1.5 hours of surgery, was notable for a plaquelike, lobular, avidly enhancing collection with MRI characteristics consistent with fluid density extending from the porus acusticus into the cerebellopontine angle. This anomalous lesion disappeared upon repeat imaging 48 hours later, and the patient had no attributable clinical sequelae. He was discharged home without issues within 12 hours of repeat imaging. We demonstrate here that immediate postoperative, gadolinium-enhanced MRI scans after tumor resection may result in avid enhancement in the region of surgical manipulation, likely due to leakage of gadolinium chelates into the subarachnoid space from residual compromise of the blood-brain barrier immediately following surgical manipulation. Early imaging is no longer routinely performed at our institution unless otherwise clinically indicated. FLAIR, fluid-attenuated inversion recoveryIAC, internal auditory canal.

Mutation spectrum and differential gene expression in cystic and solid vestibular schwannoma

We sought to characterize the mutation spectrum of NF2 and the differential gene expression in cystic and solid vestibular schwannomas. We collected tumor tissue and blood samples of 31 cystic vestibular schwannomas and 114 solid vestibular schwannomas. Mutation screening of NF2 was performed in both tumor and blood DNA samples of all patients. cDNA microarray was used to analyze the differential gene expression between 11 cystic vestibular schwannomas and 6 solid vestibular schwannomas. Expression levels of top candidate genes were verified by quantitative reverse transcription PCR. NF2 mutations were identified in 34.5% of sporadic vestibular schwannomas, with all mutations being exclusively somatic. No significant difference was found between the mutation detection rates of cystic vestibular schwannoma (35.5%) and solid vestibular schwannoma (34.2%). cDNA microarray analysis detected a total of 46 differentially expressed genes between the cystic vestibular schwannoma and solid vestibular schwannoma samples. The significantly decreased expression of four top candidate genes, C1orf130, CNTF, COL4A3, and COL4A4, was verified by quantitative reverse transcription PCR. NF2 mutations are not directly involved in the cystic formation of vestibular schwannoma. In addition, the differential gene expression of cystic vestibular schwannoma reported in our study may provide useful insights into the molecular mechanism underlying this process.

Fluid-fluid level in cystic vestibular schwannoma: a predictor of peritumoral adhesion

The aim of this study was to evaluate the clinical results and surgical outcomes of cystic vestibular schwannomas (VSs) with fluid-fluid levels. Forty-five patients with cystic VSs and 86 with solid VSs were enrolled in the study. The patients in the cystic VSs were further divided into those with and without fluid-fluid levels. The clinical and neuroimaging features, intraoperative findings, and surgical outcomes of the 3 groups were retrospectively compared. Peritumoral adhesion was significantly greater in the fluid-level group (70.8%) than in the nonfluid-level group (28.6%) and the solid group (25.6%; p < 0.0001). Complete removal of the VS occurred significantly less in the fluid-level group (45.8%, 11/24) than in the nonfluid-level group (76.2%, 16/21) and the solid group (75.6%, 65/86; p = 0.015). Postoperative facial nerve function in the fluid-level group was less favorable than in the other 2 groups; good/satisfactory facial nerve function 1 year after surgery was noted in 50.0% cases in the fluid-level group compared with 83.3% cases in the nonfluid-level group (p = 0.038). Cystic VSs with fluid-fluid levels more frequently adhered to surrounding neurovascular structures and had a less favorable surgical outcome. A possible mechanism of peritumoral adhesion is intratumoral hemorrhage and consequent inflammatory reactions that lead to destruction of the tumor-nerve barrier. These findings may be useful in predicting surgical outcome and planning surgical strategy preoperatively.

Intratumoral Haemorrhage Causing an Unusual Clinical Presentation of a Vestibular Schwannoma

We present a case of an elderly woman with no history of audiological disease with sudden onset of visual and hearing deficits associated with systemic clinical signs. On examination she had impairment of right CNs from V to X. CT and MR imaging demonstrated a cystic vestibular schwannoma with a rare intralesional fluid-fluid level correlated to a recent bleed. We include high quality MR images to show the acute impairment of the cranial nerves next to the tumour after acute bleeding. Our case report includes a voxel-based morphometry (VMB) analysis of the tumour that, as far as we know, has never been done before for such a tumour. VBM analysis was performed to calculate the hypothesized volume changes after the acute bleed which likely resulted in a sudden increase in the overall size of the tumour resulting in atypical clinical signs and symptoms due to the establishment of a mechanical conflict with the adjacent cranial nerves.

Use of apparent diffusion coefficients in evaluating the response of vestibular schwannomas to Gamma Knife surgery

Cellular density is a major factor responsible for changes in apparent diffusion coefficients (ADCs). The authors hypothesized that loss of tumor cells after Gamma Knife surgery (GKS) might alter ADC values. Magnetic resonance imaging, including diffusion-weighted (DW) imaging, was performed to detect cellular changes in brain tumors so that the authors could evaluate the tumor response to GKS as well as the efficacy of the procedure. The authors conducted a prospective trial involving 31 patients harboring solid or cystic vestibular schwannomas (VSs) that were treated with GKS. The patients underwent serial MR imaging, including DW imaging, before GKS and at multiple intervals following the procedure. The authors observed the patients over time, evaluating MR imaging findings and clinical outcomes at 6-month intervals. The ADCs were calculated from echo-planar DW images, and mean ADC values were compared at each follow-up. The mean follow-up period was 36.5 months (range 18-60 months). Imaging studies showed a reduction in tumor volume in 19 patients (61.3%) and tumor growth arrest in 9 patients (29%). In the remaining 3 patients (9.7%), tumor enlargement was documented at 18, 36, and 42 months. The mean ADC value before GKS for all solid VSs was 1.06 ± 0.17 × 10(-3) mm(2)/second, which significantly increased 6 months after GKS and continued to increase with time (p = 0.0086). The mean ADC value for treated solid tumors as of the last mean follow-up of 36 months (range 18-60 months) was 1.72 ± 0.26 × 10(-3) mm(2)/second (range 1.50-2.09 × 10(-3) mm(2)/second), which was significantly higher than that before GKS (p = 0.0001). Tumor volumes were positively related to ADC values (p = 0.03). The mean ADC value before GKS for all cystic VSs was 2.09 ± 0.24 × 10(-3) mm(2)/second (range 1.80-2.58 × 10(-3) mm(2)/second). The mean ADC value for treated cystic tumors as of the last mean follow-up of 38 months (range 18-48 months) was 1.89 ± 0.22 × 10(-3) mm(2)/second. In 3 patients harboring solid VSs, the tumor enlarged after GKS but the ADC values were higher than those before GKS. The authors considered these tumors to be controlled and continued follow-up in the patients. Apparent diffusion coefficient values may be useful for evaluating treatment results before any definite volume change is detected on imaging studies and for distinguishing radiation-induced necrosis from tumor recurrence in cases in which other imaging results are not definitive, as in cases of increased tumor volume or no volume change. The authors suggest that ADC measurements be included during routine MR imaging examinations for the evaluation of GKS results.

Removal of Large or Giant Sporadic Vestibular Schwannomas via Translabyrinthine Approach: A Report of 115 Cases

Background/Aims: The present study was a retrospective analysis of surgical outcomes of large or giant vestibular schwannomas (VSs) via a translabyrinthine approach (TLA). Methods: One hundred and fifteen sporadic large or giant VSs (maximal diameter in the cerebellopontine angle ≥31 mm) were operated via TLA between January 2001 and December 2010. We divided patients into two groups: cystic VS (CVS) and solid VS (SVS) group. The surgical outcomes were recorded and compared. Results: Total, near total, and subtotal tumor removal rate was 89.6, 7.8, and 2.6%, respectively. The near total removal rate in CVS was higher than that in SVS (16.7 vs. 3.8%). The anatomical facial nerve (FN) integrity was preserved in 101 patients (87.8%). More FNs in CVS were interrupted (22.2%). There were 29.6, 56.5, and 13.9% of patients who had a short-term (1 month) FN function of House-Brackmann grade I or II, III or IV, and V or VI, respectively. The CVS had worse FN function than the SVS group. The mortality rate was 0.9%. Cerebrospinal fluid leakage occurred in 8 patients (7.0%). Four (3.5%) patients had recurrence. Conclusions: Cyst formation is a crucial factor for surgical outcome of VS. With near total or subtotal tumor removal, worse functional outcomes in large or giant CVSs should be foreseen.

Do cystic vestibular schwannomas have worse surgical outcomes? Systematic analysis of the literature

The goal of this study was to perform a systematic quantitative comparison of the surgical outcomes between cystic vestibular schwannomas (CVSs) and solid vestibular schwannomas (SVSs). A review of English-language literature published between 1990 and 2011 was performed using various search engines including PubMed, Google Scholar, and the Cochrane database. Only studies that reported surgical results of CVSs in comparison with SVSs were included in the analysis. The primary end point of this study was surgical outcomes, defined by the following: 1) facial nerve outcomes at latest follow-up; 2) mortality rates; or 3) non-facial nerve complication index. Secondary end points included extent of resection and brainstem adherence. Nine studies comprising 428 CVSs and 1287 SVSs were included in the study. The mean age of patients undergoing surgery was 48.3 ± 6.75 and 47.1 ± 9 years for CVSs and SVSs, respectively (p = 0.8). The mean tumor diameter for CVSs was 3.9 ± 0.84 cm and that for SVSs was 3.7 ± 1.2 cm (p = 0.7). There was no significant difference in the extent of resection among CVSs and SVSs (81.2% vs 80.7%, p = 0.87) Facial nerve outcomes were significantly better in the cohort of patients with SVSs than in those with CVSs (52.1% vs 39%, p = 0.0001). The perioperative mortality rates for CVSs and SVSs were not significantly different (3% and 3.8%, respectively; p = 0.6). No significant difference was noted between the cumulative non-facial nerve complication rate (including mortality) among patients with CVSs and SVSs (24.5% and 25.6%, respectively; p = 0.75) Facial nerve outcomes are worse in patients undergoing resection for CVSs than in patients undergoing resection for SVSs. There were no significant differences in the extent of resection or postoperative morbidity and mortality rates between the cohorts of patients with vestibular schwannomas.

Extent of resection and early postoperative outcomes following removal of cystic vestibular schwannomas: surgical experience over a decade and review of the literature

Vestibular schwannomas (VSs) are benign tumors of the eighth cranial nerve sheath, representing approximately 6%-8% of all newly diagnosed brain tumors, with an annual incidence of 2000-2500 cases in the US. Although most of these lesions are solid, cystic vestibular schwannomas (CVSs) compose 4%-20% of all VSs and are commonly larger at the time of presentation. The authors present their experience with the operative management of CVSs, including surgical approach, extent of resection, and postoperative facial nerve outcomes. The literature pertaining to clinical and histopathological differences between CVSs and their solid counterparts is reviewed. The University of Southern California Department of Neurosurgery database was retrospectively reviewed to identify patients who had undergone resection of a VS between 2000 and 2010. One hundred seventy-nine patients with VS were identified. Patients with CVSs were the subject of the present analysis. Diagnosis of a CVS was made based on MRI findings. Clinical and neuroimaging data, including pre- and postoperative assessments and operative notes, were collected and reviewed. Twenty-three patients, 14 men (61%) and 9 women (39%), underwent 24 operations for CVSs. These patients composed 12.8% of all cases of VS. Patient ages ranged from 28 to 78 years (mean 55 years), and the mean maximal tumor diameter was 3.6 cm (range 2.0-4.0 cm). Patients most frequently presented with headache, hearing loss, vertigo, and dizziness. Preoperative facial numbness was reported in 44% of patients. Among the 24 cases, 13 were treated with retrosigmoid craniotomy and 11 via a translabyrinthine approach. Complete resection was achieved in 11 patients (48%), subtotal resection (STR) in 8 patients (35%), and near-total resection (NTR) in 4 patients (17%). Facial nerve outcomes were available in all except one case. Good facial nerve outcomes (House-Brackmann [HB] Grades I-III) were achieved in 82% of the patients who had undergone either NTR or STR, as compared with 73% of patients who had undergone gross-total resection (GTR; p > 0.05, Fisher exact test). In comparison, 83% of patients with solid VSs had a good HB grade (p = 0.38, Fisher exact test), although this finding did not reach statistical significance. Complications included wound infection (2 patients), delayed CSF leakage (1 patient), and a delayed temporal encephalocele following a translabyrinthine approach and requiring surgical repair (1 patient). Cystic vestibular schwannoma represents a clinical and surgical entity separate from its solid counterpart, as demonstrated by its more rapid clinical course and early surgical outcomes. Facial nerve grades may correlate with the degree of tumor resection, trending toward poorer grades with more significant resections. Although GTR is recommended whenever possible, performing an STR when facial nerve preservation is in jeopardy to improve facial nerve outcomes is the preferred strategy at the authors' institution.

A stepwise illustration of the translabyrinthine approach to a large cystic vestibular schwannoma

Of the presigmoid approaches, the translabyrinthine approach is often used when a large exposure is needed to gain access to the cerebellopontine angle but when hearing preservation is not a concern. At the authors' institution, this approach is done with the aid of ENT/otolaryngology for temporal bone drilling and exposure. In the present article and video, the authors demonstrate the use of the translabyrinthine approach for resection of a large cystic vestibular schwannoma, delineating the steps of positioning, opening, temporal bone drilling, tumor resection, and closure. Gross-total resection was achieved in the featured case. The patient's postoperative facial function was House-Brackmann Grade II on the side ipsilateral to the tumor, although function improved with time. The translabyrinthine route to the cerebellopontine angle is an excellent approach for masses that extend toward the midline or anterior to the pons. Although hearing is sacrificed, facial nerve function is generally spared.

Surgical Outcomes for Large Cystic Vestibular Schwannomas

Introduction: Cystic vestibular schwannomas (CVSs) are rare and thought to present different clinical, radiological, and treatment outcomes from their solid counterparts.

Facial nerve function after large cystic vestibular schwannomas surgery via the retrosigmoid approach

To evaluate long-term postoperative facial nerve function in patients undergoing large cystic vestibular schwannomas (VSs) surgery. Surgical outcome of 36 patients with large cystic VS was retrospectively assessed, and compared with a group of 67 patients with large solid VS. The anatomical integrity of the facial nerve was preserved in 88.9% of cystic group patients and in 92.5% of solid group patients. At 1 year after surgery, 75.8% of the patients with cystic VSs had favorable facial nerve function and 24.2% had poor function. There was no significant difference in facial nerve outcomes between cystic and solid tumors. When the cyst wall was closely adherent to the facial nerve, near total or subtotal resection was associated with a better facial nerve outcome compared to total excision (P < 0.05). In most cystic VS patients, favorable facial nerve function can be attained by surgical treatment via retrosigmoid approach. Special care should be taken to remove entire cystic VSs as well as partial cystic VSs with anterior or medial located cysts. When a dissection plane cannot be developed between the cyst wall and the facial nerve, near total or subtotal resection is of benefit for facial nerve function.

Implications of Cystic Features in Vestibular Schwannomas of Patients Undergoing Microsurgical Resection

Cystic vestibular schwannomas (VSs) are described as being more aggressive than solid tumors. We examined 468 VS patients to evaluate whether the presence of cystic components in VSs may be an important feature for predicting postoperative outcome. We selected all VS patients from a prospectively collected database (1984-2009) who underwent microsurgical resection for VS. Hearing data were analyzed using American Association of Otolaryngology-Head and Neck Surgery. Facial nerve dysfunction was analyzed using the House-Brackmann scale. We used univariate comparisons to determine the clinical impact of cystic changes on preoperative and postsurgical hearing and facial nerve preservation. We identified 58 patients (11%) with cystic changes and 410 patients with solid VSs. In this analysis, cystic VS patients tended to have larger tumors (78% of patients with >2.0 cm extrameatal extension) compared with the solid VS group, which consisted of many smaller and medium-sized tumors (P < .0001). Univariate analyses found that tumors with cystic changes did not lead to worse rates of preoperative hearing loss (χ(2), P = not significant) compared with solid VSs. Cystic changes conferred worse postoperative hearing in patients with medium-sized tumors (P = .035). Cystic changes also did not significantly affect facial nerve outcomes (χ(2), P = not significant). Cystic tumors tend to be larger than noncystic tumors and affect outcomes by reducing the rate at which hearing preservation is attempted and by worsening hearing outcome in medium-sized tumors. Further, peripheral cysts cause lower rates of hearing preservation compared with centrally located cysts.

Vestibular schwannoma of oscillating size: A case report and review of literature

Background:Vestibular schwannomas are benign brain tumors arising from the 8th cranial nerve with a varying natural history. Various reports have described discernable growth patterns for these tumors. However, growth predictability remains low because of slow and indeterminate changes over time with follow-up reports not usually exceeding 3 years. Our report describes the long-term follow-up of an unusual cystic schwannoma with growth patterns prior to and following treatment, adding valuable information to the variable natural history and outcome of these infrequent tumors.Case Description:A 68-year-old gentleman presented with a left-sided cystic vestibular schwannoma, initially managed conservatively. Imaging revealed wide variations in the size of his tumor over a period of 3 years. He was finally treated with Gamma Knife radiosurgery, and at 1 year following treatment shows tumor shrinkage with a change in tumor morphology.Conclusion:To our knowledge, the present case represents the first instance of a schwannoma showing wide fluctuations in tumor size and morphology over a period of time, with a good response to radiosurgery. We emphasize in this report that there is no “one size fits all” treatment paradigm for these tumors and each patient requires individualized care and intervention, taking into account their differing natural histories.

Schwannomes vestibulaires kystiques géants : intérêt du drainage kystopéritonéal

Introduction Surgical removal of giant cystic vestibular schwannomas is difficult because of adherences between the cyst membrane, brainstem, and the VII–VIII nerve complex. The recurrence of the cyst is frequent and requires reoperation. The aim of this study was to analyze the role of the palliative cystoperitoneal shunt in giant cystic vestibular schwannomas. Materials and methods Eighty-eight patients with a diagnosis of stage III or IV vestibular schwannoma were managed surgically from January 2000 to December 2005 in our department. Six schwannomas were deemed to be cystic according to the following criteria: a voluminous cystic component with mass effect causing symptoms as well as radiological and intraoperative identification of cystic elements. Results Complete tumor removal was achieved in two patients. After a follow-up of 5 and 7 years, these patients were asymptomatic. In four cases, we performed cyst drainage. For three patients, we implanted a permanent cystoperitoneal shunt. These patients were asymptomatic with a mean follow-up of 19.7 months. Conclusions The cystoperitoneal shunt with no valve is a valid palliative surgical solution to remove brain stem compression. Neuronavigation allows proper positioning of the drain and secures the procedure.

Cystoperitoneal shunts for cystic vestibular schwannomas

We welcome the comments made by Dagain et al. in their letter of response to our article. In particular, we note the similarity of their approach to our own with regards to cystic vestibular schwannomas in elderly patients with significant comorbidities. We agree that cyst aspiration alone is unlikely to be sufficient and that a shunt will be required in most cases. In case 1 in our paper, intermittent reservoir aspirations did however allow us to gauge the clinical response to cyst drainage before proceeding to insertion of a distal catheter. Furthermore, we very much endorse the assertion that a valve-less system is required due to tumour fluid viscosity (in both the cases we described, a valve-less system was used with a reservoir for access). We note with interest the description of the patient in whom the solid tumour increased significantly after drainage of the cystic component. This reaffirms our opinion that patients treated with this method require interval clinical and radiological assessment. The emphasis placed on neuronavigation by Dagain et al. is very much in line with our clinical practice in this area: frameless stereotaxy was used in both of the cases described. Cystoperitoneal shunting for cystic vestibular schwannomas is an extremely useful technique in specific circumstances. We thank the authors for their remarks and we await the publication of their own series with interest.

Role of cystoperitoneal shunt in the setup of cystic vestibular schwannomas drainage

Role of cystoperitoneal shunt in the setup of cystic vestibular schwannomas drainage

Cystic Vestibular Schwannoma

Review of postoperative morbidity and facial nerve outcomes of cystic vestibular schwannoma (CVS) patients compared with solid vestibular schwannoma (SVS) patients and a proposal for a new CVS classification system. Retrospective review. Tertiary care facility. Ninety-six patients with surgically treated CVS (1998-2008). Outcomes were assessed in a subpopulation of 57 patients with greater than or equal to 1-year follow-up compared with 57 SVS patients. Fifty-six CVS patients underwent the enlarged translabyrinthine approach with transapical extension (Type I), and 1 patient underwent a transcochlear/transzygomatic approach. Preoperative and postoperative (at least 1 yr) House-Brackmann facial nerve (HBFN) grade evaluation. Favorable HBFN grades (I-III) were observed in 46 (81%) CVS patients, and unfavorable HBFN grades (IV-VI) were seen in 11 (19%) CVS patients. Comparison of tumor size and 1-year HBFN grades showed significant, moderate to strong, Pearson correlation (0.38). Comparison of long-term facial nerve outcomes with a sample of 57 matched SVS patients showed no significant difference (p = 0.74). When the tumor was adherent to the facial nerve and a dissection plane could not be developed between the cyst wall and the nerve, only subtotal resection could offer the CVS patients a normal facial nerve outcome. In most CVS cases, complete resection should be foreseen. Central and thick-walled tumors can be removed in almost all cases. However, when peripheral thin-walled, adherent, cystic tumors are confronted and the cysts are medially or anteriorly located, we recommend subtotal resection, leaving portions of the cyst walls on neurovascular structures and on the facial nerve. This surgical strategy allows us to improve facial nerve outcomes and to reduce complications.

Cystic Vestibular Schwannoma Classification, Management, and Facial Nerve Outcomes

Cystic Vestibular Schwannoma Classification, Management, and Facial Nerve Outcomes

Hemorrhage into Cystic Vestibular Schwannoma Following Stereotactic Radiation Therapy

The occurrence of a hemorrhage into a vestibular schwannoma is a rare phenomenon. Several reports, however, indicate a risk profile for a subgroup of patients with vestibular schwannoma which show hypervascularity in the histopathological examination. Cystic formation, large size, mixed Antoni type and anticoagulation therapy seem to enhance the risk of tumor hemorrhage. We report on a patient with a large cystic vestibular schwannoma who died from fatal bleeding into the tumor 15 months following stereotactic radiation therapy. Since hemorrhage seems to be a relevant risk in large cystic vestibular schwannomas, a surgical treatment should be preferred whenever possible.

FLUCTUATING RESPONSE OF A CYSTIC VESTIBULAR SCHWANNOMA TO RADIOSURGERY

A vestibular schwannoma (VS) is a benign tumor of the VIIIth cranial nerve that can often be treated by microsurgery or radiosurgery and demonstrates high tumor control rates. Radiosurgery is typically performed as gamma knife surgery (GKS), although other modalities are being applied with increasing frequency. A differentiating feature in responsiveness to microsurgery or GKS is whether the VS is cystic or solid. A cystic VS is less responsive to GKS than a solid VS, representing a challenging clinical problem. GKS treatment of a cystic VS usually results in sustained expansion, sustained regression, or transient expansion followed by sustained regression. In this article, we report an atypical fluctuating course of a cystic VS after GKS, ultimately requiring surgical intervention. A 66-year-old woman presented with asymmetric hearing loss and tinnitus. Magnetic resonance imaging revealed a 2.0-cm unilateral cystic VS within the cerebellopontine angle. After GKS with a 12-Gy dose to the 50% isodose line, the tumor expanded transiently to 3.2 cm and then regressed to 1.0 cm over the next 2 years. She presented several months later with new-onset dizziness, ataxia, and facial numbness. Magnetic resonance imaging revealed a 3.2-cm multicystic VS that compressed the brainstem. After microsurgical tumor excision, the patient's symptoms abated. Our case report is a novel demonstration that a cystic VS that has regressed after GKS is still at risk for expansion. The mechanisms responsible for radiation-induced cystic tumor expansion have not been thoroughly elucidated. The risk of unpredictable tumor enlargement should be discussed with patients when considering GKS for cystic tumors.