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Clinical Neurology and Neurosurgery Aug 2023Venous hemorrhagic infarction is rare but can occur during acoustic neuroma resection [1-5]. We present the case of a 27-year-old male with 1.5 years of progressive...
Venous hemorrhagic infarction is rare but can occur during acoustic neuroma resection [1-5]. We present the case of a 27-year-old male with 1.5 years of progressive headaches, tinnitus, imbalance and hearing loss. Imaging revealed a left Koos 4 acoustic neuroma. The patient underwent a retrosigmoid approach for resection. During surgery, a vein of significant size within the capsule of the tumor was encountered and was necessary to take to proceed with resection. After coagulation of the vein, intraoperative venous congestion with cerebellar edema and hemorrhagic infarction ensued, requiring resection of a portion of the cerebellum. Given the hemorrhagic nature of the tumor, continuing tumor resection was necessary to prevent postoperative hemorrhage. This was carried out until hemostasis was achieved. 85 % resection was achieved, leaving a residual against the brainstem and cisternal course of the facial nerve. Postoperatively, the patient required 5 weeks hospitalization followed by 1 month of rehabilitation. At discharge to rehabilitation, patient had trach, PEG, left House-Brackmann 5 facial weakness, left sided deafness, and right upper extremity hemiparesis (1/5). At 7 months follow up, he continued to have left House-Brackmann 5 facial weakness and left sided deafness but trach and PEG had been removed and strength had improved to 5/5. We demonstrate in this video the unfortunate and rare occurrence of intraoperative venous hemorrhagic infarction during acoustic neuroma resection - particularly for large tumors in young patients - and discuss its etiology and surgical steps that are necessary to partially remedy its devastating impact on the patient. The patient consented to the procedure and participating in this surgical video.
Topics: Male; Humans; Adult; Neuroma, Acoustic; Hyperemia; Facial Nerve; Facial Paralysis; Hemorrhage; Brain Stem Infarctions; Deafness; Brain Stem; Retrospective Studies
PubMed: 37320888
DOI: 10.1016/j.clineuro.2023.107827 -
Journal of Neurological Surgery Reports Oct 2023Vestibular schwannomas (VSs) are treated with microsurgery and/or radiosurgery. Repeat resection is rare, and few studies have reported postoperative outcomes. The...
Vestibular schwannomas (VSs) are treated with microsurgery and/or radiosurgery. Repeat resection is rare, and few studies have reported postoperative outcomes. The objective of this study was to describe clinical characteristics and outcomes in patients undergoing repeat surgery for VS. All adult (≥ 18 years) patients undergoing VS resection between 2003 and 2022 at our institution were retrospectively reviewed to identify patients who underwent repeat surgery of an ipsilateral VS following prior gross-total (GTR) or subtotal resection. Patient, radiographic, and clinical characteristics were reviewed. Primary outcomes were postoperative tumor volume, extent of resection, postoperative cranial nerve deficits, and time to further tumor progression. Of 102 patients undergoing VS resection, 6 (5.9%) had undergone repeat surgery. Median (range) follow-up was 20 (5-117) months. Three patients were female. Median age was 56 (36-60) years. Median pre- and postoperative tumor volumes were 8.2 (1.8-28.2) cm and 0.4 (0-3.8) cm . GTR was achieved in two patients. Four patients had higher House-Brackmann scores at last follow-up, but none had tumor progression. In this small cohort of patients, repeat resection of recurrent or progressive VS can effectively reduce tumor volume with acceptable perioperative outcomes.
PubMed: 37900579
DOI: 10.1055/s-0043-1776124 -
British Journal of Neurosurgery Oct 2023Malignant change in vestibular schwannoma is rare and intracranial metastatic deposits have not been reported.
BACKGROUND
Malignant change in vestibular schwannoma is rare and intracranial metastatic deposits have not been reported.
CLINICAL PRESENTATION
We report the case of a 64 year old woman with a benign vestibular schwannoma who underwent translabyrinthine excision in 1996 and Gamma Knife radiosurgery (Elekta AB, Stockholm, Sweden) (GKRS) in 2006. She presents 10 years after GKRS with progressive neurological deterioration. Histopathologic analysis confirms a malignant peripheral nerve sheath tumour, WHO grade IV with subsequent metastatic spread to the left thalamus confirmed on biopsy.
CONCLUSION
We report a rare case of a vestibular schwannoma metastasizing with histological confirmation. It also reminds us of malignant conversion of a benign vestibular schwannoma following GKRS and subsequent aggressive behaviour, with poor prognosis.
Topics: Female; Humans; Middle Aged; Neuroma, Acoustic; Radiosurgery
PubMed: 33686895
DOI: 10.1080/02688697.2021.1884650 -
Science Advances Nov 2023Vestibular schwannoma (VS) is an intracranial tumor arising from neoplastic Schwann cells and typically presenting with hearing loss. The traditional belief that hearing...
Vestibular schwannoma (VS) is an intracranial tumor arising from neoplastic Schwann cells and typically presenting with hearing loss. The traditional belief that hearing deficit is caused by physical expansion of the VS, compressing the auditory nerve, does not explain the common clinical finding that patients with small tumors can have profound hearing loss, suggesting that tumor-secreted factors could influence hearing ability in VS patients. We conducted profiling of patients' plasma for 66 immune-related factors in patients with sporadic VS ( > 170) and identified and validated candidate biomarkers associated with tumor size (S100B) and hearing (MCP-3). We further identified a nine-biomarker panel (TNR-R2, MIF, CD30, MCP-3, IL-2R, BLC, TWEAK, eotaxin, and S100B) with outstanding discriminatory ability for VS. These findings revealed possible therapeutic targets for VS, providing a unique diagnostic tool that may predict hearing change and tumor growth in VS patients, and may inform the timing of tumor resection to preserve hearing.
Topics: Humans; Neuroma, Acoustic; Hearing Loss; Hearing; Biomarkers; Deafness
PubMed: 37948527
DOI: 10.1126/sciadv.adf7295 -
Brain Impairment : a Multidisciplinary... Jan 2024Background Acoustic neuromas (ANs) are consistently associated with decreased quality of life (QOL) related to the physical and psychosocial impacts of symptoms...
Background Acoustic neuromas (ANs) are consistently associated with decreased quality of life (QOL) related to the physical and psychosocial impacts of symptoms experienced from the tumour and its treatment. This study explored patient-reported experiences of ANs in New Zealand, with a focus on the impact on QOL and the provision of information, support and services. Methods A mixed methods approach was taken, conducting an online community survey that included the Penn Acoustic Neuroma Quality of Life Scale (N = 52). Those who indicated interest were offered semi-structured interviews after the survey (N = 17), which were analysed using content analysis. Results A negative impact on QOL was found, highlighting five key themes in the experiences of people: (1) ongoing physical, social and psychological impacts; (2) information and support from the medical system; (3) autonomy and decision-making; (4) the importance of peer support; and (5) remaining positive - life goes on. Conclusions Our findings indicate areas for improvement that may benefit people's healthcare experience and QOL. Both quantitative and qualitative results identified gaps associated with person-centred care and the need for information, education, emotional support and access to services. Recommendations include a need for more information (verbal and written) during all stages of diagnosis and treatment, shared decision-making and increased access to allied health, including psychological services and support groups.
Topics: Humans; Neuroma, Acoustic; Quality of Life; Self-Help Groups; New Zealand
PubMed: 38566292
DOI: 10.1071/IB23072 -
World Neurosurgery Jul 2023Improved technology in vestibular neuroma resection and facial nerve protection has become more sophisticated, and the protection of hearing during vestibular schwannoma...
BACKGROUND
Improved technology in vestibular neuroma resection and facial nerve protection has become more sophisticated, and the protection of hearing during vestibular schwannoma resection is crucial. Currently, brainstem auditory evoked potential (BAEP), cochlear electrography, and cochlear nerve compound action potential (CNAP) are frequently used. The CNAP waveform is stable; however, the recording electrode can easily affect the procedure and cannot map the auditory nerve. The purpose of the study was to explore a simple method to record the CNAP and map the auditory nerve.
METHODS
In this study, CNAP was recorded using a facial nerve bipolar stimulator to localize and protect the auditory nerve. The BAEP click stimulation mode was used. A bipolar stimulator was used as the recording electrode to record CNAP and locate anatomical displacement of the auditory nerve. The CNAP of 40 patients was monitored. Pure tone audiometry, speech discrimination score, and auditory evoked potential (BAEP) evaluations were performed on all patients before and after surgery.
RESULTS
Of the 40 patients, 30 patients obtained CNAP during surgery, and the rate of CNAP obtained was significantly higher than that of BAEP. The sensitivity and specificity of decrease in CNAP in predicting significant hearing loss were 88.9% and 66.7%, respectively. The sensitivity and specificity of the disappearance of CNAP in predicting significant hearing loss were 52.9% and 92.3%, respectively.
CONCLUSIONS
The bipolar facial nerve stimulator can locate and protect the auditory nerve by recording a stable potential. The CNAP obtained rate was significantly higher than that of BAEP. The disappearance of BAEP during acoustic neuroma monitoring can be used as a standard alert for the surgeon, and decrease in CNAP is an alert for the operator.
Topics: Humans; Neuroma, Acoustic; Action Potentials; Facial Nerve; Cochlear Nerve; Evoked Potentials, Auditory, Brain Stem; Deafness
PubMed: 37030482
DOI: 10.1016/j.wneu.2023.03.146 -
Journal of Korean Medical Science Oct 2023Since the long-term outcomes of 162 patients who underwent gamma knife radiosurgery (GKS) as an initial or adjuvant treatment for acoustic neuromas (ANs) with unilateral...
BACKGROUND
Since the long-term outcomes of 162 patients who underwent gamma knife radiosurgery (GKS) as an initial or adjuvant treatment for acoustic neuromas (ANs) with unilateral hearing loss were first reported in 1998, there has been no report of a comprehensive analysis of what has changed in GKS practice.
METHODS
We performed a retrospective study of the long-term outcomes of 106 patients with unilateral sporadic ANs who underwent GKS as an initial treatment. The mean patient age was 50 years, and the mean initial tumor volume was 3.68 cm (range, 0.10-23.30 cm). The median marginal tumor dose was 12.5 Gy (range, 8.0-15.0 Gy) and the median follow-up duration was 153 months (range, 120-216 months).
RESULTS
The tumor volume increased in 11 patients (10.4%), remained stationary in 27 (25.5%), and decreased in 68 patients (64.2%). The actuarial 3, 5, 10, and 15-year tumor control rates were 95.3 ± 2.1%, 94.3 ± 2.2%, 87.7 ± 3.2%, and 86.6 ± 3.3%, respectively. The 10-year actuarial tumor control rate was significantly lower in the patients with tumor volumes of ≥ 8 cm ( = 0.010). The rate of maintaining the same Gardner-Robertson scale grade was 28.6%, and that of serviceable hearing was 46.4%. The rates of newly developed facial and trigeminal neuropathy were 2.8% and 4.7%, respectively. The patients who received marginal doses of less than 12 Gy revealed higher tumor control failure rates ( = 0.129) and newly occurred facial or trigeminal neuropathy rates ( = 0.040 and 0.313, respectively).
CONCLUSION
GKS as an initial treatment for ANs could be helpful in terms of tumor control, the preservation of serviceable hearing, and the prevention of cranial neuropathy. It is recommended to perform GKS as soon as possible not only for tumor control in unilateral ANs with hearing loss but also for hearing preservation in those without hearing loss.
Topics: Humans; Middle Aged; Neuroma, Acoustic; Radiosurgery; Retrospective Studies; Follow-Up Studies; Hearing Loss; Trigeminal Nerve Diseases; Treatment Outcome
PubMed: 37846791
DOI: 10.3346/jkms.2023.38.e332 -
British Journal of Neurosurgery Aug 2023We present a case of spontaneous 79% regression in the size of a vestibular schwannoma (VS). A 41-year-old lady with a large (36 mm) vestibular schwannoma underwent...
We present a case of spontaneous 79% regression in the size of a vestibular schwannoma (VS). A 41-year-old lady with a large (36 mm) vestibular schwannoma underwent serial follow-up MRI scans which demonstrated the shrinkage over 9 years; the highest relative spontaneous regression recorded in the literature for vestibular schwannoma of this size.
Topics: Female; Humans; Adult; Neuroma, Acoustic; Radiosurgery; Magnetic Resonance Imaging
PubMed: 30829550
DOI: 10.1080/02688697.2019.1582749 -
Neuro-oncology Aug 2023Prospective data on maintenance therapy with bevacizumab for persons with NF2-related schwannomatosis (NF2-SWN) is lacking. In this prospective multicenter phase II...
BACKGROUND
Prospective data on maintenance therapy with bevacizumab for persons with NF2-related schwannomatosis (NF2-SWN) is lacking. In this prospective multicenter phase II study, we evaluated the efficacy, safety, and tolerability of bevacizumab for maintenance therapy in children and adults with NF2-SWN and hearing loss due to vestibular schwannomas (VS).
METHODS
Following induction therapy, participants received bevacizumab 5 mg/kg every 3 weeks for 18 months. Participants were monitored for changes in hearing, tumor size, and quality of life (QOL), and for adverse events. Hearing loss was defined as a statistically significant decline in word recognition score (WRS) or pure-tone average compared to the study baseline; tumor growth was defined as >20% increase in volume compared to baseline.
RESULTS
Twenty participants with NF2-SWN (median age 23.5 years; range, 12.5-62.5 years) with hearing loss in the target ear (median WRS 70%, range 2%-94%) received maintenance bevacizumab. Freedom from hearing loss in the target ear was 95% after 48 weeks, 89% after 72 weeks, and 70% after 98 weeks. Freedom from tumor growth in the target VS was 94% after 48 weeks, 89% after 72 weeks, and 89% after 98 weeks. NF2-related QOL remained stable for 98 weeks whereas tinnitus-related distress decreased. Maintenance bevacizumab was well tolerated, with 3 participants (15%) discontinuing treatment due to adverse events.
CONCLUSIONS
Maintenance bevacizumab (5 mg/kg every 3 weeks) is associated with high rates of hearing and tumor stability during 18 months of follow-up. No new unexpected adverse events related to bevacizumab were identified in this population.
Topics: Adult; Child; Humans; Young Adult; Neuroma, Acoustic; Bevacizumab; Quality of Life; Prospective Studies; Treatment Outcome; Neurofibromatosis 2; Hearing Loss
PubMed: 37010875
DOI: 10.1093/neuonc/noad066 -
Journal of Neuro-oncology Oct 2023The immortality of cancer cells relies on maintaining the length of telomeres, which prevents cellular senescence and enables unlimited replication. However, little is...
BACKGROUND
The immortality of cancer cells relies on maintaining the length of telomeres, which prevents cellular senescence and enables unlimited replication. However, little is currently known about telomerase activity and the alternative lengthening of telomeres (ALT) in vestibular schwannomas. In this study we aimed to elucidate the role that telomerase and ALTs play in vestibular schwannomas.
METHODS
To address this gap, we conducted a study where we used the gene set variation analysis algorithm with bulk RNA-seq and single-cell RNA-seq to identify the characteristics of each group of patients with vestibular schwannomas, based on their telomere maintenance mechanism subtype.
RESULTS
Our findings suggest that patients with relatively high ALT-like groups have a better prognosis than those with relatively high telomerase groups. Specifically, we found that the high telomerase group had relatively higher antigen-presenting cell (APC) activity than the high ALT like group. At the single-cell level, microglia, neutrophils, and fibroblasts showed high telomerase activity and relatively high APC activity compared to other cell types. In addition, Schwann cells in the group with low ALT levels exhibited elevated immune activity at the single-cell level.
CONCLUSION
These results suggest that personalized drug therapy could be developed from the perspective of precision medicine for patients with relatively high telomerase activity and a high ALT-like group.
Topics: Humans; Telomerase; Neuroma, Acoustic; Telomere; Telomere Homeostasis
PubMed: 37864645
DOI: 10.1007/s11060-023-04458-5