Two-Stage Surgical Management for Acutely Presented Large Vestibular Schwannomas: Report of Two Cases
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PROVIDER: S-EPMC10669422 | biostudies-literature | 2023 Nov
REPOSITORIES: biostudies-literature
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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:Objective Large vestibular schwannomas rarely present in pregnant women. Diagnosis and management of these tumors during pregnancy present a therapeutic challenge. Methods A 20-year-old primigravida woman at 26 weeks' gestation was transferred to our facility with gait imbalance, left facial weakness, left ear hearing loss, and recent nausea and vomiting. Magnetic resonance imaging revealed a large left cerebellopontine angle mass with extension into the left internal auditory canal and compression of the fourth ventricle resulting in mild hydrocephalus. The patient was admitted with a plan for early delivery at 32 weeks followed by tumor resection. One week later, the patient's headache and neurologic symptoms worsened due to increased hydrocephalus; a ventriculoperitoneal shunt was placed. The next day, an emergent cesarean delivery was performed due to worsening respiratory status. Four days later, a tracheostomy and percutaneous endoscopic gastrostomy tube were placed due to dysphagia. Eight days after the delivery, the mass was resected with a left retrosigmoid approach without complications. Immunohistochemistry confirmed vestibular cellular schwannoma on cranial nerve VIII showing unusually high mitotic activity. Results The patient was discharged to inpatient rehabilitation on postoperative day 12 without new neurologic deficit. At 1 month, the patient was swallowing without aspiration. Her facial sensation had returned, her facial weakness remained stable, and her gait was significantly improved. Conclusion If the patient is neurologically stable, the best option is to delay resection until after delivery. If resection is necessary during pregnancy, the optimal time is during the second trimester.
| S-EPMC4078191 | biostudies-other
Project description:Right atrial masses raised pose 3 major possibilities including tumors, thrombi, or vegetations. We present 2 cases: first, a 34-year-old male with no medical history, who presented with dyspnea, pleuritic pain, and fever; and the second, 65-year-old male with similar symptoms and a history of a left renal carcinoma. Both patients had right atrial masses found on a transthoracic echocardiogram. Cardiac magnetic resonance imaging and an 18 FDG-PET were necessary finding thrombi in the first patient; and tumoral thrombi in the second one. A multimodality imaging approach to right atrial masses is essential for proper diagnosis and therapeutic decision-making.
| S-EPMC9389139 | biostudies-literature
Project description:ImportanceHigh surgical vestibular schwannoma case volume in a medical institution may decrease the risk of adverse outcomes among patients undergoing vestibular schwannoma surgery.ObjectiveTo study the association between surgical vestibular schwannoma case volume and excess time in the hospital after vestibular schwannoma surgery.Design, setting, and participantsThis cohort study evaluated data from the National Cancer Database from January 1, 2004, through December 31, 2019, on Commission on Cancer-accredited facilities in the US. The hospital-based sample comprised adult patients aged 18 years or older with a vestibular schwannoma treated with surgery.ExposuresFacility case volume, defined as the mean number of surgical vestibular schwannoma cases per year in the 2 years preceding the index case.Main outcomes and measuresThe primary outcome was a composite of prolonged hospital stay (>90th percentile) or 30-day readmission. Risk-adjusted restricted cubic splines were used to model the probability of the outcome according to facility volume. The inflection point (in cases per year) when the declining risk of excess time in the hospital began to plateau was selected as the threshold to define high- and low-volume facilities. Outcomes were compared among patients treated at high- and low-volume facilities, with mixed-effects logistic regression models adjusting for patient sociodemographic characteristics, comorbidities, tumor size, and clustering within facilities. Collected data were analyzed between June 24 and August 31, 2022.ResultsAmong 11 524 eligible patients (mean [SD] age, 50.2 [12.8] years; 53.5% female; 46.5% male) who underwent surgical resection of vestibular schwannoma at 66 reporting facilities, the median length of stay was 4 (IQR, 3-5) days, and 655 patients (5.7%) were readmitted within 30 days. The median case volume was 16 (IQR, 9-26) cases per year. An adjusted restricted cubic spline model identified a downtrending probability of excess time in the hospital with increasing volume. The declining risk of excess time in the hospital began to plateau at a facility volume of 25 cases per year. Surgery at a facility with an annual case volume at or above this threshold was independently associated with a 42% reduction in the odds of excess time in the hospital compared with surgery at a low-volume center (odds ratio, 0.58; 95% CI, 0.44-0.77).Conclusions and relevanceThis cohort study found that among adults undergoing vestibular schwannoma surgery, a higher facility case volume was associated with a reduced risk of prolonged hospital stay or 30-day readmission. A facility case volume of 25 cases per year may represent a risk-defining threshold.
| S-EPMC9982737 | biostudies-literature
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:Schwannoma is a type of tumor originating from Schwann cells of peripheral nerves. In this study, we report a rare case of two giant connected retroperitoneal schwannomas. The patient presented to our department with a 1-day history of abdominal pain and without other symptoms. There were no abnormalities in the patient's tumor markers. Abdominal plain computed tomography (CT) revealed two (combined) retroperitoneal masses appearing as soft tissue-density shadows with uneven internal density, cystic low-density shadows, and patchy calcification shadows. The larger mass measured approximately 12.0 cm × 12.3 cm in size. The tumors were completely excised by a reasonable surgical approach while the surrounding organs closely related to the tumor were preserved. Postoperative pathology confirmed that the tumors were benign schwannomas. In the 18-month follow-up, the patient had no recurrences and was asymptomatic. We summarize the diagnosis and treatment of two rare combined giant retroperitoneal schwannomas in a single patient. Laparotomy for the management of retroperitoneal giant schwannomas may be safe and effective.
| S-EPMC9434686 | biostudies-literature
Project description:Introduction Large vestibular aqueduct syndrome (LVAS) is characterized by the enlargement of the vestibular aqueduct associated with sensorineural hearing loss. It is the most common radiographically detectable inner ear anomaly in congenital hearing loss. LVAS may occur as an isolated anomaly or in association with other inner ear malformations. Objective To report three cases of isolated LVAS with a focus on preoperative assessment, surgical issues, and short-term postoperative follow-up with preliminary auditory habilitation outcomes. Resumed Report One girl and two boys with LVAS were assessed and cochlear implantation was performed for each. Various ways of intraoperative management of cerebrospinal fluid gusher and postoperative care and outcomes are reported. Conclusion Cochlear implantation in the deaf children with LVAS is feasible and effective.
| S-EPMC4593917 | biostudies-other
Project description:Schwannomas are benign tumors in 95% of cases and very rarely occur in the retroperitoneum. We report the cases of a 35-year-old man with abdominal discomfort and a 50-year-old asymptomatic woman with large retroperitoneal masses. Both underwent multivisceral surgery to exclude an adrenal carcinoma, and the pathologic diagnosis showed schwannomas in both cases. Despite morphological imaging, it was not possible to get a clear diagnosis preoperatively.
| S-EPMC7798304 | biostudies-literature