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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 -
Neuro-oncology Practice Aug 2021Large vestibular schwannomas (VS) pose a treatment challenge for both microsurgery (MS) and stereotactic radiosurgery (SRS). Technical developments have allowed for... (Review)
Review
BACKGROUND
Large 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.
METHODS
PubMed 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.
RESULTS
Twenty-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 ( < .01), 0.47 ( = .05), and 0.60 ( = .22); for facial nerve impairment, these ORs were 1.08 ( = .69), 3.45 ( = .28), and 0.87 ( = .71), respectively.
CONCLUSIONS
The 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.
PubMed: 34277019
DOI: 10.1093/nop/npab011 -
European Archives of... May 2023Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases... (Review)
Review
PURPOSE
Intralabyrinthine schwannomas (ILSs) are an uncommon finding. Diagnosis is challenging and no gold standard treatment exists yet. In this article, we present a two-cases series and review the latest available literature to assess the best diagnostic and therapeutic scheme.
METHODS
We reviewed the latest available literature assessing most frequent and relevant sets of symptoms, clinical features of the disease, diagnostic tests and imaging, possible treatments and after-surgery hearing rehabilitation techniques. We then compared literature data to our own series ones.
RESULTS
ILSs clinical presentation and development may overlap with other, more common otological conditions. Full audiometric battery test, electrophysiological study of VEMPS and MRI with contrast enhancement all appear to be critical to correctly diagnose these tumors. Several treatments exist: radiological follow-up, radiation therapy, full or partial surgical excision. Hearing rehabilitation is mostly accomplished through simultaneous cochlear implantation.
CONCLUSIONS
Our case-series data matches the available literature. ILSs are a rare type of vestibular schwannomas. Diagnosis in challenging and delayed in time as all the diagnostic tests, yet sensitive, are not specific for ILSs. The most suitable treatment seems to be surgical excision of these tumors followed by simultaneous cochlear implantation to restore hearing.
Topics: Humans; Neurilemmoma; Neuroma, Acoustic; Hearing; Hearing Tests; Hearing Loss, Sensorineural
PubMed: 36648549
DOI: 10.1007/s00405-023-07823-2 -
Frontiers in Aging Neuroscience 2022Acoustic neuroma (AN) is a common benign tumor. Little is known of neuropsychological studies in patients with acoustic neuroma, especially cognitive neuropsychology,...
OBJECTIVE
Acoustic neuroma (AN) is a common benign tumor. Little is known of neuropsychological studies in patients with acoustic neuroma, especially cognitive neuropsychology, and the neuropsychological abnormalities of patients affect their life quality. The purpose of this study was to explore the changes in the cognitive function of patients with acoustic neuroma, and the possible mechanism of these changes by structural magnetic resonance imaging.
MATERIALS AND METHODS
We used a neuropsychological assessment battery to assess cognitive function in 69 patients with acoustic neuroma and 70 healthy controls. Then, we used diffusion tensor imaging data to construct the structural brain network and calculate topological properties based on graph theory, and we studied the relation between the structural brain network and cognitive function. Moreover, three different subnetworks (short-range subnetwork, middle-range subnetwork, and long-range subnetwork) were constructed by the length of nerve fibers obtained from deterministic tracking. We studied the global and local efficiency of various subnetworks and analyzed the correlation between network metrics and cognitive function. Furthermore, connectome edge analysis directly assessed whether there were differences in the number of fibers in the different brain regions. We analyzed the relation between the differences and cognitive function.
RESULTS
Compared with the healthy controls, the general cognitive function, memory, executive function, attention, visual space executive ability, visual perception ability, movement speed, and information processing speed decreased significantly in patients with acoustic neuroma. A unilateral hearing loss due to a left acoustic neuroma had a greater impact on cognitive function. The results showed that changes in the global and local metrics, the efficiency of subnetworks, and cognitively-related fiber connections were associated with cognitive impairments in patients with acoustic neuroma.
CONCLUSION
Patients exhibit cognitive impairments caused by the decline of the structure and function in some brain regions, and they also develop partial compensation after cognitive decline. Cognitive problems are frequent in patients with acoustic neuroma. Including neuropsychological aspects in the routine clinical evaluation and appropriate treatments may enhance the clinical management and improve their life quality.
PubMed: 36389069
DOI: 10.3389/fnagi.2022.970159 -
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 -
La Clinica Terapeutica May 2021Over the process of establishing the causal relation-ship, medical and legal methodologies may be at variance over the definitional standards and terminologies applied,...
Can a causal relationship be established between acoustic neuroma and occupational exposure to non-ionizing radiations from mobile devices? Comparison between scientific literature data and medico-legal methodology.
BACKGROUND
Over the process of establishing the causal relation-ship, medical and legal methodologies may be at variance over the definitional standards and terminologies applied, which can hinder the activities of expert witnesses.
OBJECTIVES
The article's authors have set out to assess whether, and under what conditions, a causal relationship can be established between acoustic neuroma and exposure to non-ionizing radiation from mobile communication devices.
METHODS
The study design is a Systematic Review. The authors have drawn upon a 2020 Turin Court of Appeals ruling which found such a causal relationship in a somewhat peculiar case: rare tumor and exposure to non-ionizing radiation of unusually long and regular dura-tion. The case presents several peculiarities, herein analyzed in light of a) scientific evidence relative to the etiopathogenesis of the neuroma; b) available medico-legal literature defining causality evaluation criteria, and lastly c) court filings in regard to the probability standards applied to prove causal relationship.
RESULTS
A direct tie cannot be proven, not even in cases of sub-stantially intense and lengthy exposure, if the medico-legal standards applied are not consistent enough to prove that nexus is more likely than not.
DISCUSSION
Several elements suggest a causal relationship is unlikely: a) a dearth of evidence on humans; b) rats exposed to such radiation have developed cardiac tumors, not in their ears; c) exposure has caused no tumors in mice; d) the length of exposure is incompat-ible with tumor size and type. That fourth point only concerns the case herein explored, whereas the first three have a general scope of validity. The main limitation of the present study design is the heterogeneity among the included studies. Retrospective and prospective studies have been included, which may be a source of bias.
Topics: Animals; Causality; Computers, Handheld; Humans; Mice; Neuroma, Acoustic; Occupational Exposure; Radiation, Nonionizing; Rats; Risk Factors
PubMed: 33956036
DOI: 10.7417/CT.2021.2313 -
Brazilian Journal of Otorhinolaryngology 2023To review the literature on the diagnosis and treatment of vestibular schwannoma. (Review)
Review
OBJECTIVE
To review the literature on the diagnosis and treatment of vestibular schwannoma.
METHODS
Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions.
RESULTS
The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy.
CONCLUSIONS
Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.
Topics: Humans; Neuroma, Acoustic; Brazil; Societies, Medical; Advisory Committees; Otolaryngology; Radiosurgery; Magnetic Resonance Imaging
PubMed: 37813009
DOI: 10.1016/j.bjorl.2023.101313 -
BMJ Case Reports Mar 2021Hypnic headache (HH) is a rare, primary headache syndrome that invariably occurs during sleep and wakes the patient. Acoustic neuroma (AN) is a benign tumour that...
Hypnic headache (HH) is a rare, primary headache syndrome that invariably occurs during sleep and wakes the patient. Acoustic neuroma (AN) is a benign tumour that uncommonly presents with isolated headache. Here, we describe a patient with AN that presented with an HH-like syndrome. A 40-year-old woman presented with 4 months of generalised, throbbing, nocturnal headaches that woke her from sleep. Neurological examination was unremarkable. Retrospectively, she reported a 4-year history of mild, bilateral tinnitus. Neuroimaging demonstrated a large, left-sided AN in the cerebellopontine angle without obstructive hydrocephalus. Gamma knife radiosurgery controlled tumour growth. One year after radiosurgery, she became nocturnal headache-free. AN has not previously been described as presenting with an HH-like syndrome. There are four previous reports of an HH-like syndrome secondary to intracranial masses. In all cases, patients became headache-free following surgery. This advocates for neuroimaging to exclude structural causes.
Topics: Adult; Female; Headache; Headache Disorders, Primary; Humans; Neuroma, Acoustic; Retrospective Studies; Sleep
PubMed: 33687931
DOI: 10.1136/bcr-2020-235830 -
Frontiers in Neurology 2022Previous studies have shown that patients with acoustic neuroma (AN) sometimes present with sudden sensorineural hearing loss (SSNHL) as an initial symptom. The purpose...
BACKGROUND
Previous studies have shown that patients with acoustic neuroma (AN) sometimes present with sudden sensorineural hearing loss (SSNHL) as an initial symptom. The purpose of this research was to investigate the clinical characteristics, diagnosis, and treatment of AN in patients initially diagnosed with SSNHL.
MATERIALS AND METHODS
We reviewed retrospectively the medical records of all patients who were treated as SSNHL initially and were later diagnosed with AN after undergoing magnetic resonance imaging (MRI) at our hospital between 2008 and 2021. Patient demographics, associated complaints (mostly tinnitus and vertigo), the severity of hearing loss, audiogram configurations, auditory brainstem response (ABR), and MRI examination were reviewed and analyzed. In addition, treatment outcomes and management protocols were also included in this study.
RESULTS
A total of 10 (0.7%, 10/1,383) patients presented with SSNHL as the initial symptom and were diagnosed as AN by MRI finally. Of the 10 patients enrolled in this study, four were men and six were women. The average age at the time of diagnosis of SSNHL was 46.2 ± 13.16 years. These patients exhibited varying severity of hearing loss and a variety of audiogram configurations. All patients showed an abnormal ABR. According to the Koos grading standard, there were 5 grade I (intracanalicular [IAC]) tumors, 3 grade II tumors, and 2 grade III tumors. The treatment outcome revealed that 2 patients exhibited recovery of the average hearing of impaired frequency by more than 15 dB, and 6 patients showed no recovery. Furthermore, four patients were referred to undergo surgical treatment after being diagnosed with AN, 1 patient accepted stereotactic radiation therapy, and the remaining 5 patients were on a "wait and scan" strategy.
CONCLUSION
The hearing loss of patients with AN presented with SSNHL may improve with drug treatment. Hearing recovery for SSNHL does not exclude the presence of AN, and all patients initially diagnosed with SSNHL should undergo MRI and ABR to prevent misdiagnosis and delays in potential treatment.
PubMed: 36061993
DOI: 10.3389/fneur.2022.953265 -
Neuro-oncology Practice Dec 2021Differences in long-term outcomes of single-fraction stereotactic radiosurgery (SRS) between gamma knife (GK) and linear accelerator (LINAC) systems for vestibular... (Review)
Review
BACKGROUND
Differences in long-term outcomes of single-fraction stereotactic radiosurgery (SRS) between gamma knife (GK) and linear accelerator (LINAC) systems for vestibular schwannoma (VS) management remain unclear. To investigate differences in safety and efficacy between modalities, we conducted a meta-analysis of studies over the past decade.
METHODS
MEDLINE, EMBASE, and Cochrane databases were queried for studies with the following inclusion criteria: English language, published between January 2010 and April 2020, cohort size ≥30, and mean/median follow-up ≥5 years. Odds ratios (OR) compared rates of tumor control, hearing preservation, and cranial nerve toxicities before and after SRS.
RESULTS
Thirty-nine studies were included (29 GK, 10 LINAC) with 6516 total patients. Tumor control rates were 93% (95% CI 91-94%) and 94% (95% CI 91-97%) for GK and LINAC, respectively. Both GK (OR 0.06, 95% CI 0.02-0.13) and LINAC (OR 0.47, 95% CI 0.29-0.76) reduced odds of serviceable hearing. Neither GK (OR 0.71, 95% CI 0.41-1.22) nor LINAC (OR 1.13, 95% CI 0.64-2.00) impacted facial nerve function. GK decreased odds of trigeminal nerve (TN) impairment (OR 0.55, 95% CI 0.32-0.94) while LINAC did not impact TN function (OR 1.45, 95% CI 0.81-2.61). Lastly, LINAC offered decreased odds of tinnitus (OR 0.15, 95% CI 0.03-0.87) not observed with GK (OR 0.70, 95% CI 0.48-1.01).
CONCLUSIONS
VS tumor control and hearing preservation rates are comparable between GK and LINAC SRS. GK may better preserve TN function, while LINAC decreases tinnitus rates. Future studies are warranted to investigate the efficacy of GK and LINAC SRS more directly.
PubMed: 34777833
DOI: 10.1093/nop/npab052