Large Vestibular Schwannomas Presenting during Pregnancy: Management Strategies.
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ABSTRACT:
SUBMITTER: Shah KJ
PROVIDER: S-EPMC4078191 | biostudies-other | 2014 Jun
REPOSITORIES: biostudies-other
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Project description:The study was conducted to analyze outcomes following surgical management of large and giant vestibular schwannomas and management options for residual disease. This retrospective case note study includes patients who had undergone microsurgical resection of sporadic, large, or giant vestibular schwannomas from 1986 to 2008. Tumors are classified as large if the largest extracanalicular diameter was 3.5 cm or greater and giant if 4.5 cm or greater. The study included 45 patients (33 large, 12 giant tumors), mean tumor size 4.1 cm. Total excision was achieved in 14 cases (31.1%), near-total in 26 (57.8%), and subtotal in 5 (11.1%). Facial nerve outcome was House-Brackmann Grade I/II in 25 cases (55.6%), III/IV in 16 (35.6%), and V/VI in 4 (8.9%). No recurrence has been detected in those undergoing a complete resection. No residual tumor growth been observed in 15 of 26 who underwent near-total resection (57.7%). Of 11 patients, 10 received further treatment as their residual tumors showed growth. In the subtotal excision group, one patient died, three have demonstrated no growth, and one residual tumor has grown slightly but not required intervention. Optimal management for patients with large or giant vestibular schwannomas has yet to be determined. Management decisions must balance long term function with tumor control.
| S-EPMC3312131 | biostudies-other
Project description:Trigeminal schwannomas (TS), though the second most common intracranial schwannomas, represent only 0.8 to 8% of all Schwannomas. Advancement in imaging and microsurgical techniques has led to a remarkable improvement in the outcome of these benign tumors. Multicompartmental TS, though extensive, have an excellent outcome after surgery. In this article, we present our experience in the management of multicompartmental TS (types middle/posterior [MP], middle/extracranial [ME], and middle/posterior and extracranial [MPE]) and outcome in this rather uncommon group of tumors. This retrospective study included all the cases of multicompartmental TS operated at our institute from 1999 to 2009. The medical data were analyzed retrospectively. The demographic profile, clinical features, radiological findings, management strategies, postoperative complications, length of hospitalization, and outcome were noted. Follow-up data were collected from outpatient department records. The range and average duration of follow-up were noted. There were a total of 43 patients with TS operated over this period. Among them, 4 were type B, 5 type C, 11 type D, 18 type E, and 5 type F. The study included 26 patients (4 type B, 18 type E, and 4 type B). A variety of approaches were used to approach the tumor. Of 26, 23 patients had a gross total or near-total excision while 2 patients were lost to follow-up. Among the three patients who had a near-total excision and follow-up magnetic resonance imaging showed a small residual tumor, two are on close follow-up with no increase in the size of the tumor over a follow-up period of 3 years, the other patient is a 5-year-old boy who is too young for radiosurgery and is on follow-up. There was no mortality while four patients have had fresh permanent postoperative deficits. Multicompartmental TS are a rare, complex but eminently treatable group of tumors. A variety of surgical approaches can be used to excise the tumor. The choice of approach needs to be individualized with total excision providing excellent results.
| S-EPMC3312128 | biostudies-other
Project description:Vestibular Schwannomas are benign neoplasms that arise from the vestibular nerve. The hallmark of these tumors is the biallelic inactivation of NF2. Transcriptomic alterations, such as the Nrg1/ErbB2 pathway, have been described in Schwannomas. Here, we have performed a whole transcriptomic analysis in 31 vestibular Schwannomas and 9 control nerves in the Affymetrix Gene 1.0ST platform, validated by quantitative Real-Time PCR using TaqMan Low Density Arrays. We performed a mutational analysis of NF2 by PCR/dHPLC and MLPA as well as a microsatellite marker analysis of the loss of heterozygosity of chromosome 22q. The microarray analysis showed that 1516 genes were deregulated, and 48 of the genes were validated by qRT-PCR. At least two genetic hits (allelic loss and/or gene mutation) in NF2 were found in 16 tumors, seven cases showed one hit and eight tumors showed no NF2 alteration. As conclusion, MET and associated genes such as ITGA4/B6, PLEXNB3/SEMA5 and CAV1 showed a clear deregulation in vestibular Schwannomas. In addition, androgen receptor (AR) downregulation may denote a hormonal effect or cause in this tumor. Furthermore, the osteopontin gene (SPP1), which is involved in Merlin protein degradation, was upregulated, which suggests that this mechanism may also exert a pivotal role in Schwannoma Merlin depletion. Finally, no major differences were found between tumors of different sizes, histological types or NF2 status, which suggests that at the mRNA level all Schwannomas, regardless of molecular and clinical characteristics, may share common features that can be used in the fight against them. In order to find target to fight against vestibular schwannoma, we performed an analysis of gene expression by microarrays.
2013-03-01 | E-GEOD-39645 | biostudies-arrayexpress
Project description:Vestibular Schwannomas are benign neoplasms that arise from the vestibular nerve. The hallmark of these tumors is the biallelic inactivation of NF2. Transcriptomic alterations, such as the Nrg1/ErbB2 pathway, have been described in Schwannomas. Here, we have performed a whole transcriptomic analysis in 31 vestibular Schwannomas and 9 control nerves in the Affymetrix Gene 1.0ST platform, validated by quantitative Real-Time PCR using TaqMan Low Density Arrays. We performed a mutational analysis of NF2 by PCR/dHPLC and MLPA as well as a microsatellite marker analysis of the loss of heterozygosity of chromosome 22q. The microarray analysis showed that 1516 genes were deregulated, and 48 of the genes were validated by qRT-PCR. At least two genetic hits (allelic loss and/or gene mutation) in NF2 were found in 16 tumors, seven cases showed one hit and eight tumors showed no NF2 alteration. As conclusion, MET and associated genes such as ITGA4/B6, PLEXNB3/SEMA5 and CAV1 showed a clear deregulation in vestibular Schwannomas. In addition, androgen receptor (AR) downregulation may denote a hormonal effect or cause in this tumor. Furthermore, the osteopontin gene (SPP1), which is involved in Merlin protein degradation, was upregulated, which suggests that this mechanism may also exert a pivotal role in Schwannoma Merlin depletion. Finally, no major differences were found between tumors of different sizes, histological types or NF2 status, which suggests that at the mRNA level all Schwannomas, regardless of molecular and clinical characteristics, may share common features that can be used in the fight against them.
2013-03-01 | GSE39645 | GEO
Project description:BackgroundLarge vestibular schwannomas (VS) pose a treatment challenge for both microsurgery (MS) and stereotactic radiosurgery (SRS). Technical developments have allowed for safer irradiation of large tumors. It remains unclear if SRS can achieve appropriate tumor control and acceptable cranial nerve toxicities. In this study, we assess outcomes of irradiation for large VS.MethodsPubMed MEDLINE, EMBASE, Web of Science, and Cochrane were searched for all the studies assessing SRS outcome in large VS. Primary endpoints included clinical and radiographic tumor control, need for salvage surgery, serviceable hearing, cranial nerve V and VII impairment, presence of hydrocephalus requiring shunting, and presence of vertigo/dizziness.ResultsTwenty-two studies were identified that met selection criteria for analysis from an initial pool of 1272 reports. They were evaluated according to treatment protocol: 1) single-dose SRS (13 studies, 483 patients), 2) combination of MS and SRS (7 studies, 182 patients), and 3) fractionated SRS (3 studies, 82 patients). Tumor control was achieved in 89%, 94%, and 91% of patients, respectively. Odds ratios (ORs) of post- over pretreatment serviceable hearing were 0.42 (P < .01), 0.47 (P = .05), and 0.60 (P = .22); for facial nerve impairment, these ORs were 1.08 (P = .69), 3.45 (P = .28), and 0.87 (P = .71), respectively.ConclusionsThe management of large VS remains challenging. All treatment modalities resulted in high tumor control rates and worsening of pretreatment hearing. None, however, caused significant facial nerve impairment, suggesting that management strategies incorporating focal irradiation can be successful.
| S-EPMC8278356 | biostudies-literature
Project description:The presenting symptoms of the tumor suppressor gene syndrome neurofibromatosis type 2 (NF2) are often non-specific and unrelated to the disease hallmark bilateral vestibular schwannomas (VS). However, age at onset and presenting symptoms may have predictive values for the clinical course of VS. In this retrospective single-center study, we addressed this issue by reviewing 106 patients with 194 VS. Presenting symptoms attributable to VS commonly occur in 87% of adults and 31% of children. Age at onset significantly correlates with tumor volumes at presentation (p = 0.034). In addition, age at onset significantly correlates with pure-tone average (p = 0.0001), speech discrimination scores (p = 0.001), age at beginning of hearing loss (p = 0.0001), age at deafness (p = 0.0001), and age at first surgery (p = 0.0001). Patients presenting with VS related symptoms had significantly (p < 0.05) worse hearing values at presentation and after surgery. These patients also exhibited higher growth rates and tumor volumes compared to patients with non-VS related presenting symptoms, but this difference did not reach the significance level of p < 0.05. Due to the late appearance of these symptoms, the time of beginning hearing loss, surgery and deafness is significantly delayed (p < 0.05) compared to patients not presenting with VS. In summary, age at onset and type of presenting symptom provide excellent prognostic parameters for predicting VS- and hearing-related clinical course.
| S-EPMC7563356 | biostudies-literature
Project description:Vestibular schwannomas (VSs) grow in the region where the energy from mobile phone use is absorbed. We examined the associations of VSs with mobile phone use. This study included 119 patients who had undergone surgical tumor removal. We used two approaches in this investigation. First, a case-control study for the association of mobile phone use and incidence of VSs was conducted. Both cases and controls were investigated with questions based on INTERPHONE guidelines. Amount of mobile phone use according to duration, daily amount, and cumulative hours were compared between two groups. We also conducted a case-case study. The location and volume of the tumors were investigated by MRI. Associations between the estimated amount of mobile phone use and tumor volume and between the laterality of phone use and tumor location were analyzed. In a case-control study, the odds ratio (OR) of tumor incidence according to mobile phone use was 0.956. In the case-case study, tumor volume and estimated cumulative hours showed a strong correlation (r(2)?=?0.144, p?=?0.002), and regular mobile phone users showed tumors of a markedly larger volume than those of non-regular users (p?<?0.001). When the analysis was limited to regular users who had serviceable hearing, laterality showed a strong correlation with tumor side (OR?=?4.5). We found that tumors may coincide with the more frequently used ear of mobile phones and tumor volume that showed strong correlation with amount of mobile phone use, thus there is a possibility that mobile phone use may affect tumor growth.
| S-EPMC3907669 | biostudies-literature
Project description:BackgroundThe choice of an appropriate strategy for intracanalicular vestibular schwannoma (ICVS) is still debated. We conducted a systematic review and meta-analysis with the aim to compare treatment outcomes amongst management strategies (conservative surveillance (CS), microsurgical resection (MR), or stereotactic radiosurgery (SRS)) aiming to inform guideline recommendations on behalf of the International Stereotactic Radiosurgery Society (ISRS).MethodsUsing PRISMA guidelines, we reviewed manuscripts published between January 1990 and October 2021 referenced in PubMed or Embase. Inclusion criteria were peer-reviewed clinical studies or case series reporting a cohort of ICVS managed with CS, MR, or SRS. Primary outcome measures included tumor control, the need for additional treatment, hearing outcomes, and posttreatment neurological deficits. These were pooled using meta-analytical techniques and compared using meta-regression with random effect.ResultsForty studies were included (2371 patients). The weighted pooled estimates for tumor control were 96% and 65% in SRS and CS series, respectively (P < .001). Need for further treatment was reported in 1%, 2%, and 25% for SRS, MR, and CS, respectively (P = .001). Hearing preservation was reported in 67%, 68%, and 55% for SRS, MR, and CS, respectively (P = .21). Persistent facial nerve deficit was reported in 0.1% and 10% for SRS and MR series, respectively (P = .01).ConclusionsSRS is a noninvasive treatment with at least equivalent rates of tumor control and hearing preservation as compared to MR, with the caveat of better facial nerve preservation. As compared to CS, upfront SRS is an effective treatment in achieving tumor control with similar rates of hearing preservation.
| S-EPMC10912008 | biostudies-literature
Project description:Background Spontaneous hemorrhage into vestibular schwannomas (VSs) is rare and can render more rapid symptom onset and a seemingly poorer prognosis for an otherwise benign pathology. We describe our series of hemorrhagic VS (HVSs) and systematically reviewed the literature to better understand relevant clinical factors and outcomes. Methods Retrospective case review series and systematic review of the literature using PRISMA guidelines. Results Fifty-three patients with HVS met inclusion criteria. Compared with historical data for all VS, patients with HVS had relatively higher rates of perioperative mortality, significant preoperative facial weakness, and harbored relatively larger tumors. Regardless of the extent of resection (EOR), surgery for HVS resulted in significant improvement of facial weakness ( p = 0.041), facial numbness ( p < 0.001), vertigo ( p < 0.001), and headache ( p < 0.001). Patients with facial weakness tended to have larger tumors ( p = 0.058) on average and demonstrated significant improvement after surgery, irrespective of EOR ( p < 0.01). The use of blood-thinning medications did not affect patient health outcome. Histopathology of HVS samples showed an increased number of dilated/ectatic thin-walled vascular channels, reflective of potentially increased vascular permeability and hypervascularity. Conclusion HVS may be an aggressive subgroup of VS, associated with a surprisingly high mortality rate. When features of HVS are identified on imaging, these patients should be treated expeditiously, especially given that facial nerve dysfunction, which is identified in more than half of patients with HVS, appears to be reversible. Overall, this study has significant implications in the management of VS, raising awareness of a small, but highly morbid subgroup.
| S-EPMC8289498 | biostudies-literature
Project description:Vestibular schwannomas (VSs) are the most common tumours of the cerebellopontine angle. Ninety-five percent of people with VS present with sensorineural hearing loss (SNHL); the mechanism of this SNHL is currently unknown. To establish the first model to study the role of VS-secreted factors in causing SNHL, murine cochlear explant cultures were treated with human tumour secretions from thirteen different unilateral, sporadic VSs of subjects demonstrating varied degrees of ipsilateral SNHL. The extent of cochlear explant damage due to secretion application roughly correlated with the subjects' degree of SNHL. Secretions from tumours associated with most substantial SNHL resulted in most significant hair cell loss and neuronal fibre disorganization. Secretions from VSs associated with good hearing or from healthy human nerves led to either no effect or solely fibre disorganization. Our results are the first to demonstrate that secreted factors from VSs can lead to cochlear damage. Further, we identified tumour necrosis factor alpha (TNFα) as an ototoxic molecule and fibroblast growth factor 2 (FGF2) as an otoprotective molecule in VS secretions. Antibody-mediated TNFα neutralization in VS secretions partially prevented hair cell loss due to the secretions. Taken together, we have identified a new mechanism responsible for SNHL due to VSs.
| S-EPMC4686978 | biostudies-literature