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Pediatric Neurology Sep 2022The neurofibromatoses comprise three different genetic conditions causing considerable morbidity and mortality: neurofibromatosis type 1 (NF1), neurofibromatosis type 2... (Review)
Review
INTRODUCTION
The neurofibromatoses comprise three different genetic conditions causing considerable morbidity and mortality: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). This review summarizes recent and ongoing clinical trials involving patients with neurofibromatoses to better understand the current state of clinical trial research centered around these conditions and inform areas of need.
METHODS
A search was conducted using the Cochrane Central Register of Controlled Trials and clinicaltrials.gov databases. Inclusion and exclusion criteria were designed to identify clinical trials focused on patients with NF1, NF2, or SWN completed in or after 2010 and in process as of December 31, 2021. Information was collected using standardized guidelines.
RESULTS
A total of 134 clinical trials were included, with 75 (56%) completed and 59 (44%) in process. For completed trials, 74% (n = 56) involved patients with NF1, and of those based on specific tumors (n = 26, 46%), the majority focused on plexiform neurofibromas (PNs) (n = 12, 46%). For ongoing trials, 79% (n = 47) involve patients with NF1, and of those based on specific tumors (n = 29, 61%), the majority are focused on PNs (n = 13, 45%).
CONCLUSION
Both recent and ongoing clinical trials have primarily focused on patients with NF1 and the treatment of PNs. This research has led to the first FDA-approved drug for NF1-PN and has changed management of these tumors, allowing for systemic therapy rather than reliance on only a surgical modality. Trials evaluating comorbid psychiatric conditions and quality of life among patients with any of the neurofibromatoses appear less common. These areas may warrant focus in future studies to improve clinical management.
Topics: Humans; Neurilemmoma; Neurofibroma, Plexiform; Neurofibromatoses; Neurofibromatosis 1; Neurofibromatosis 2; Quality of Life; Skin Neoplasms
PubMed: 35759947
DOI: 10.1016/j.pediatrneurol.2022.06.003 -
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 -
Clinical Neurology and Neurosurgery May 2023Secondary trigeminal neuralgia is a facial pain in trigeminal nerve dermatome caused by an underlying disease, such as cerebellopontine angle tumours. Treatment options... (Review)
Review
BACKGROUND
Secondary trigeminal neuralgia is a facial pain in trigeminal nerve dermatome caused by an underlying disease, such as cerebellopontine angle tumours. Treatment options to relieve the pains were surgical tumour resection and stereotactic radiosurgery of the tumour or trigeminal nerve. This study aims to review the efficacy of open surgery and stereotactic radiosurgery and recommend the treatment of choice for secondary trigeminal neuralgia due to cerebellopontine angle tumours.
METHOD
The inclusion criteria were studies covering patients with trigeminal neuralgia associated with cerebellopontine angle tumours that were treated with either open surgery or stereotactic radiosurgery and reported pain outcomes after treatment. Non-English articles or studies with a population of less than five were excluded. We systematically searched studies from PubMed, Ebscohost, and Cochrane Library from inception until December 20, 2021. Several works of literature from manual search were also added. Selected articles were appraised using a critical appraisal tool for prognostic studies.
RESULT
Included articles were 26 retrospective studies and one prospective study comprising 517 patients. Of 127 schwannomas, 226 epidermoids, 154 meningiomas, and ten other tumours, 320 cases received surgical tumour excision with or without MVD, 196 had tumour-targeted radiosurgery, and 22 underwent nerve-targeted radiosurgery. In surgical series, 92.2 % gained pain improvement, 2.8 % were unchanged, and 4.5 % had recurrence; none of the patients had worsened outcomes. In cases treated with tumour-targeted radiosurgery, the improvement rate was 79.1 %, unchanged at 14.3 %, recurrence at 26.5 %, and worse symptoms rate after the intervention was 6.6 %. Six patients with recurrent pain after tumour-targeted radiosurgery received secondary nerve-targeted radiosurgery with improved outcomes. Only one patient in our review underwent primary nerve-targeted radiosurgery, and the result was satisfactory. One study treated 15 patients with a single session of tumour-targeted and nerve-targeted radiosurgery, with an improvement rate of 93.3 % and a recurrence rate of 21.4 %.
CONCLUSION
Open surgery releasing the nerve root from compressive lesions is advocated to be the first-line treatment to gain satisfactory outcomes. Total removal surgery is recommended if possible. Nerve-targeted radiosurgery should be reserved as a secondary treatment for recurrent cases.
Topics: Humans; Trigeminal Neuralgia; Neuroma, Acoustic; Radiosurgery; Treatment Outcome; Retrospective Studies; Prospective Studies; Facial Pain; Meningeal Neoplasms
PubMed: 37001475
DOI: 10.1016/j.clineuro.2023.107683 -
Systematic Reviews Dec 2022Vestibular schwannomas are benign tumours for which various treatments are available. We performed a systematic review of prospective controlled trials comparing the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Vestibular schwannomas are benign tumours for which various treatments are available. We performed a systematic review of prospective controlled trials comparing the patient-relevant benefits and harms of single-fraction stereotactic radiosurgery (sfSRS) with microsurgical resection (MR) in patients with vestibular schwannoma.
METHODS
We searched for randomized controlled trials (RCTs) and non-randomized prospective controlled trials in MEDLINE, Embase, the Cochrane Central Register of Controlled Trials, and study registries (last search: 09/2021) and also screened reference lists of relevant systematic reviews. Manufacturers were asked to provide unpublished data. Eligible studies investigated at least one patient-relevant outcome. We assessed the risk of bias (high or low) at the study and outcome level. If feasible, meta-analyses were performed. We graded the results into different categories (hint, indication, or proof of greater benefit or harm).
RESULTS
We identified three non-randomized prospective controlled trials of generally low quality with evaluable data on 339 patients with unilateral vestibular schwannoma. There was an indication of greater benefit of sfSRS compared with MR for facial palsy (OR 0.06, 95% CI 0.02-0.21, p < 0.001, 2 studies), hearing function (no pooled estimate available, 2 studies), and length of hospital stay (no pooled estimate available, 2 studies). We found no clinically relevant differences for mortality, vertigo, headaches, tinnitus, balance function, work disability, adverse events, and health-related quality of life.
CONCLUSIONS
Our systematic review indicates that sfSRS has greater benefits than MR in patients with unilateral vestibular schwannoma. However, it is unclear whether this conclusion still holds after 2 years, as long-term studies are lacking. It is also unclear whether the effects of sfSRS are similar in patients with bilateral vestibular schwannomas. Long-term prospective studies including patients with this condition would therefore be useful.
SYSTEMATIC REVIEW REGISTRATION
The full (German language) protocol and report (Commission No. N20-03) are available on the institute's website: www.iqwig.de/en/projects/n20-03.html.
Topics: Humans; Microsurgery; Neuroma, Acoustic; Radiosurgery; Treatment Outcome; Controlled Clinical Trials as Topic
PubMed: 36503553
DOI: 10.1186/s13643-022-02118-9 -
Wideochirurgia I Inne Techniki... Sep 2022Intracanalicular vestibular schwannomas (IVS) account for 8% of all vestibular schwannomas and their detection is still increasing due to high availability of magnetic... (Review)
Review
Intracanalicular vestibular schwannomas (IVS) account for 8% of all vestibular schwannomas and their detection is still increasing due to high availability of magnetic resonance (MRI). Radiosurgery is one of several commonly acceptable methods of IVS treatment, but some risk may still exist with that treatment. The aim of this study is to analyze the clinical outcomes in tumor control and hearing preservation after radiosurgery of IVS. The retrospective analysis included 14 scientific papers available in the PubMed database. Assessment of tumor volume was performed based on gadolinium-enhanced T1-weighted scans. Hearing preservation was assessed using the Gardner-Robertson classification (GR class). Statistical analysis was performed using IBM SPSS Statistics 27. It was revealed that tumor growth control in IVS treated with radiosurgery was higher than in the wait-and-see strategy. The hearing preservation was similar in patients after wait and see and the surgical group. Radiosurgery was associated with low risk of facial nerve dysfunction.
PubMed: 36187055
DOI: 10.5114/wiitm.2022.115169 -
Frontiers in Surgery 2022Survival amongst posterior fossa tumour (PFT) patients is improving. Clinical endpoints such as overall survival fail to depict QoL. There is yet to be a review of... (Review)
Review
INTRODUCTION
Survival amongst posterior fossa tumour (PFT) patients is improving. Clinical endpoints such as overall survival fail to depict QoL. There is yet to be a review of current QoL instruments used for adult PFTs. Aim of this review is to outline the QoL reporting in the management of PFTs and measure participation level.
METHODS
This systematic review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis. A search strategy to identify adult patients with PFTs who took part in QoL metrics was conducted. Observational and experimental studies published from 1990 to date were included. Studies with a sample size less than 10 and performance measures such as Karnofsky Performance Status were not considered.
RESULTS
A total of 116 studies were included in the final analysis. Vestibular schwannomas were the most common tumour pathology ( = 23,886, 92.6%) followed by pilocytic astrocytomas ( = 657, 2.5%) and meningiomas ( = 437, 1.7%) Twenty-five different QoL measures were used in the study pool. SF-36 was the most common ( = 55, 17 47.4%) QoL metric in the whole study pool, followed by the Penn Acoustic Neuroma QoL scale ( = 24, 20.7%) and Dizziness Handicap Inventory ( = 16, 13.8%). Seventy-two studies reported less-than 100% participation in QoL evaluation. The commonest reason for non-participation was a lack of response ( = 1,718, 60.8%), incomplete questionnaires ( = 268, 9.4%) and cognitive dysfunction ( = 258, 9.1%).
CONCLUSION
Informed clinical decision-making in PFT patients requires the development of specific QoL outcomes. Core outcome sets, and minimal clinically important differences (MCID) are essential for these metrics to show clinically significant improvements in patient QoL.
PubMed: 36303860
DOI: 10.3389/fsurg.2022.970889 -
International Journal of Occupational... Feb 2017Results of epidemiological studies on the association between use of mobile phone and brain cancer are ambiguous, as well as the results of 5 meta-analysis studies... (Meta-Analysis)
Meta-Analysis Review
Results of epidemiological studies on the association between use of mobile phone and brain cancer are ambiguous, as well as the results of 5 meta-analysis studies published to date. Since the last meta-analysis (2009), new case-control studies have been published, which theoretically could affect the conclusions on this relationship. Therefore, we decided to perform a new meta-analysis. We conducted a systematic review of multiple electronic data bases for relevant publications. The inclusion criteria were: original papers, case-control studies, published till the end of March 2014, measures of association (point estimates as odds ratio and confidence interval of the effect measured), data on individual exposure. Twenty four studies (26 846 cases, 50 013 controls) were included into the meta-analysis. A significantly higher risk of an intracranial tumor (all types) was noted for the period of mobile phone use over 10 years (odds ratio (OR) = 1.324, 95% confidence interval (CI): 1.028-1.704), and for the ipsilateral location (OR = 1.249, 95% CI: 1.022-1.526). The results support the hypothesis that long-term use of mobile phone increases risk of intracranial tumors, especially in the case of ipsilateral exposure. Further studies are needed to confirm this relationship. Int J Occup Med Environ Health 2017;30(1)27-43.
Topics: Brain Neoplasms; Case-Control Studies; Cell Phone; Electromagnetic Fields; Environmental Exposure; Humans; Salivary Gland Neoplasms
PubMed: 28220905
DOI: 10.13075/ijomeh.1896.00802 -
Journal of Neuro-oncology Oct 2023To perform a systematic review of literature specific to single-fraction stereotactic radiosurgery (SRS) for large vestibular schwannomas (VS), maximum... (Review)
Review
Single-fraction radiosurgery outcomes for large vestibular schwannomas in the upfront or post-surgical setting: a systematic review and International Stereotactic Radiosurgery Society (ISRS) Practice Guidelines.
PURPOSE
To perform a systematic review of literature specific to single-fraction stereotactic radiosurgery (SRS) for large vestibular schwannomas (VS), maximum diameter ≥ 2.5 cm and/or classified as Koos Grade IV, and to present consensus recommendations on behalf of the International Stereotactic Radiosurgery Society (ISRS).
METHODS
The Medline and Embase databases were used to apply the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) approach. We considered eligible prospective and retrospective studies, written in the English language, reporting treatment outcomes for large VS; SRS for large post-operative tumors were analyzed in aggregate and separately.
RESULTS
19 of the 229 studies initially identified met the final inclusion criteria. Overall crude rate of tumor control was 89% (93.7% with no prior surgery vs 87.7% with prior surgery). Rates of salvage microsurgical resection, need for shunt, and additional SRS in all series versus those with no prior surgery were 9.6% vs 3.3%, 4.7% vs 6.4% and 1% vs 0.9%, respectively. Rates of facial palsy and hearing preservation in all series versus those with no prior surgery were 1.3% vs 3.4% and 34.2% vs 40.4%, respectively.
CONCLUSIONS
Upfront SRS resulted in high rates of tumor control with acceptable rates of facial palsy and hearing preservation as compared to the results in those series including patients with prior surgery (level C evidence). Therefore, although large VS are considered classic indication for microsurgical resection, upfront SRS can be considered in selected patients and we recommend a prescribed marginal dose from 11 to 13 Gy (level C evidence).
Topics: Humans; Radiosurgery; Retrospective Studies; Neuroma, Acoustic; Prospective Studies; Facial Paralysis; Treatment Outcome; Follow-Up Studies
PubMed: 37843727
DOI: 10.1007/s11060-023-04455-8 -
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 determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery. (Review)
Review
OBJECTIVES
To determine the cut-off point of the cochlear radiation dose as a risk factor for hearing loss in patients with vestibular schwannoma treated with radiosurgery.
METHODS
A systematic review of the literature was performed without language or publication year restrictions in the MEDLINE/PubMed, EMBASE, Web of Science, LILACS/VHL and Cochrane Library databases. Studies that met the following criteria were included: 1) population: adults of both sexes who underwent radiosurgery for vestibular schwannoma treatment; 2) exposure: cochlear radiation; 3) outcome: hearing loss; 4) type of study: cohort. Two independent reviewers conducted the entire review process. The registration number in PROSPERO was CRD42020206128.
RESULTS
From the 333 articles identified in the searches, seven were included after applying the eligibility criteria. There was no standardization as to how to measure exposure or outcome in the included studies, and most studies did not present sufficient data to enable meta-analysis.
CONCLUSION
It was not possible to determine a cut-off point for high cochlear dose that could be considered a risk factor for hearing loss.
Topics: Adult; Female; Humans; Male; Deafness; Hearing Loss; Neuroma, Acoustic; Radiation Dosage; Radiosurgery; Retrospective Studies; Treatment Outcome
PubMed: 37579571
DOI: 10.1016/j.bjorl.2023.101300