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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 -
Dental Research Journal 2017Intraoral ancient schwannoma is a rare type of oral schwannoma, which is encapsulated and well demarcated from the surrounding tissues. Ancient schwannomas are... (Review)
Review
Intraoral ancient schwannoma is a rare type of oral schwannoma, which is encapsulated and well demarcated from the surrounding tissues. Ancient schwannomas are associated with conventional features of neurilemmoma; however, they are distinguished from other types of schwannoma due to factors such as the long history, cellular architecture showing hypocellularity, and hyalinized matrices. This systematic review was performed through searching in databases such as PubMed and Google Scholar using related keywords (intraoral, oral, ancient, schwannoma, and neurilemmoma). Eventually, 26 case reports were systematically reviewed by the researchers. Required data were extracted by one researcher, and all the selected articles were reviewed in full text after screening. This systematic review aimed to determine the most significant influential factors in intraoral ancient schwannoma and evaluate the diagnostic and therapeutic methods in this regard.
PubMed: 28584531
DOI: No ID Found -
Neurosurgery Jun 2023Stereotactic radiosurgery (SRS) is one of the main treatment options in the management of small to medium size vestibular schwannomas (VSs), because of high tumor... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Stereotactic radiosurgery (SRS) is one of the main treatment options in the management of small to medium size vestibular schwannomas (VSs), because of high tumor control rate and low cranial nerves morbidity. Series reporting long-term hearing outcome (>3 years) are scarce.
OBJECTIVE
To perform a systematic review of the literature and meta-analysis, with the aim of focusing on long-term hearing preservation after SRS.
METHODS
Using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we reviewed articles published between January 1990 and October 2020 and referenced in PubMed or Embase. Inclusion criteria were peer-reviewed clinical study or case series of VSs treated with SRS (single dose), reporting hearing outcome after SRS with a median or mean audiometric follow-up of at least 5 years. Hearing preservation, cranial nerves outcomes, and tumor control were evaluated.
RESULTS
Twenty-three studies were included. Hearing preservation was found in 59.4% of cases (median follow-up 6.7 years, 1409 patients). Main favorable prognostic factors were young age, good hearing status, early treatment after diagnosis, small tumor volume, low marginal irradiation dose, and maximal dose to the cochlea. Tumor control was achieved in 96.1%. Facial nerve deficit and trigeminal neuropathy were found in 1.3% and 3.2% of patients, respectively, both significantly higher in Linear Accelerator series than Gamma Knife series ( P < .05).
CONCLUSION
Long-term hearing preservation remains one of the main issues after SRS, with a major impact on health-related quality of life. Our meta-analysis suggests that hearing preservation can be achieved in almost 60% of patients after a median follow-up of 6.7 years, irrespective of the technique.
Topics: Humans; Neuroma, Acoustic; Radiosurgery; Treatment Outcome; Quality of Life; Hearing; Follow-Up Studies; Retrospective Studies
PubMed: 36735500
DOI: 10.1227/neu.0000000000002354 -
Indian Journal of Otolaryngology and... Dec 2022To evaluate the clinico-epidemiological aspects, pathological features, diagnostic methods, management protocol and functional outcome of the intra-parotid facial nerve...
To evaluate the clinico-epidemiological aspects, pathological features, diagnostic methods, management protocol and functional outcome of the intra-parotid facial nerve schwannoma (IFNS) and to present a case report on intra parotid facial nerve schwannoma. PubMed, ProQuest, Google scholar, Science direct and Scopus were screened for studies. Article selection and data extraction was done by one investigator and other investigator confirmed its accuracy. After abstract and text screening a total of 69 articles were finally selected for the study with the inclusion and exclusion criteria of the systematic review as per PRISMA guidelines. With addition of one case reported to our department. The mean age of diagnosis was 43 ± 16 years with a slight female predominance. The mean duration of the tumour was 29.5 months and the mean size of the tumour on initial diagnosis was 3.6 ± 1.67 cm. Pleomorphic adenoma was the primary diagnosis in 44 cases. Superficial parotidectomy was done in 64 cases followed by resection in 47 cases. Reconstructive treatment was carried out by an end-to-end anastomosis in 3 patients and by facial-hypoglossal anastomosis in 16 patients, GAN cable grafting in 5 patients, a greater auricular nerve graft was done in18 patients and end-to-side interposed sural nerve graft in 8 patients. The type D tumours are treated by extended resection of the facial nerve, which is difficult to reconstruct and also employs a nerve graft that does not often give acceptable recovery of facial function. Facial nerve schwannomas being a rare entity poses a dilemma in diagnosis and management. Managing the lesions is also difficult as intraoperative adherence to the nerve makes a tumour free margin difficult without sacrificing the nerve. At present there is no consensus regarding the management of various types of intra-parotid facial nerve shwannoma.
PubMed: 36742919
DOI: 10.1007/s12070-021-03013-w -
Neurosurgical Review Jul 2023Proton beam therapy is considered, by some authors, as having the advantage of delivering dose distributions more conformal to target compared with stereotactic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Proton beam therapy is considered, by some authors, as having the advantage of delivering dose distributions more conformal to target compared with stereotactic radiosurgery (SRS). Here, we performed a systematic review and meta-analysis of proton beam for VSs, evaluating tumor control and cranial nerve preservation rates, particularly with regard to facial and hearing preservation.
METHODS
We reviewed, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) articles published between 1968 and September 30, 2022. We retained 8 studies reporting 587 patients.
RESULTS
Overall rate of tumor control (both stability and decrease in volume) was 95.4% (range 93.5-97.2%, p heterogeneity= 0.77, p<0.001). Overall rate of tumor progression was 4.6% (range 2.8-6.5%, p heterogeneity < 0.77, p<0.001). Overall rate of trigeminal nerve preservation (absence of numbness) was 95.6% (range 93.5-97.7%, I = 11.44%, p heterogeneity= 0.34, p<0.001). Overall rate of facial nerve preservation was 93.7% (range 89.6-97.7%, I = 76.27%, p heterogeneity<0.001, p<0.001). Overall rate of hearing preservation was 40.6% (range 29.4-51.8%, I = 43.36%, p heterogeneity= 0.1, p<0.001).
CONCLUSION
Proton beam therapy for VSs achieves high tumor control rates, as high as 95.4%. Facial rate preservation overall rates are 93%, which is lower compared to the most SRS series. Compared with most currently reported SRS techniques, proton beam radiation therapy for VSs does not offer an advantage for facial and hearing preservation compared to most of the currently reported SRS series.
Topics: Humans; Neuroma, Acoustic; Proton Therapy; Hearing; Cranial Nerves; Facial Nerve; Radiosurgery; Treatment Outcome; Follow-Up Studies; Retrospective Studies
PubMed: 37402894
DOI: 10.1007/s10143-023-02060-x -
Yonsei Medical Journal May 2016Many epidemiological studies have investigated environmental risk factors for the development of acoustic neuroma. However, these results are controversial. We conducted... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Many epidemiological studies have investigated environmental risk factors for the development of acoustic neuroma. However, these results are controversial. We conducted a meta-analysis of case-control studies to identify any potential relationship between history of noise exposure, smoking, allergic diseases, and risk of acoustic neuroma.
MATERIALS AND METHODS
We searched PubMed to identify relevant articles. Two researchers evaluated the eligibility and extracted the data independently.
RESULTS
Eleven case-control studies were included in our meta-analysis. Acoustic neuroma was found to be associated with leisure noise exposure [odds ratio (OR)=1.33, 95% confidence interval (CI): 1.05-1.68], but not with occupational noise exposure and ever noise exposure (OR=1.20, 95% CI: 0.84-1.72 and OR=1.15, 95% CI: 0.80-1.65). The OR of acoustic neuroma for ever (versus never) smoking was 0.53 (95% CI: 0.30-0.94), while the subgroup analysis indicated ORs of 0.95 (95% CI: 0.81-1.10) and 0.49 (95% CI: 0.41-0.59) for ex-smoker and current smoker respectively. The ORs for asthma, eczema, and seasonal rhinitis were 0.98 (95% CI: 0.80-1.18), 0.91 (95% CI: 0.76-1.09), and 1.52 (95% CI: 0.90-2.54), respectively.
CONCLUSION
Our meta-analysis is suggestive of an elevated risk of acoustic neuroma among individuals who were ever exposed to leisure noise, but not to occupational noise. Our study also indicated a lower acoustic neuroma risk among ever and current cigarette smokers than never smokers, while there was no significant relationship for ex-smokers. No significant associations were found between acoustic neuroma and history of any allergic diseases, such as asthma, eczema, and seasonal rhinitis.
Topics: Adult; Asthma; Environmental Exposure; Female; Humans; Hypersensitivity; Leisure Activities; Neuroma, Acoustic; Noise; Occupational Exposure; Risk Factors; Smoking
PubMed: 26996581
DOI: 10.3349/ymj.2016.57.3.776 -
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 -
Current Oncology (Toronto, Ont.) Jul 2022Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed... (Review)
Review
BACKGROUND
Craniovertebral junction (CVJ) schwannomas are rare, with surgery and stereotactic radiosurgery (SRS) being effective yet challenging options. We systematically reviewed the literature on CVJ schwannomas.
METHODS
PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA statement to include studies reporting CVJ schwannomas. Clinical features, management, and outcomes were analyzed.
RESULTS
We collected 353 patients from 101 included articles. Presenting symptoms were mostly neck pain (30.3%) and headache (26.3%), with most cranial neuropathies involving the XII (31.2%) and X (24.4%) nerves. Most tumors originated from C2 (30.9%) and XII (29.4%) nerves, being extracranial (45.1%) and intradural-extradural (44.2%). Erosion of C1-C2 vertebrae (37.1%), the hypoglossal canal (28.3%), and/or jugular foramen (20.1%) were noted. All tumors were operated, preferably with the retrosigmoid approach (36.5%), with the far-lateral approach (29.7%) or with the posterior approach and cervical laminectomy (26.9%), far-lateral approaches (14.2%), or suboccipital craniotomy with concurrent cervical laminectomy (14.2%). Complete tumor resection was obtained most frequently (61.5%). Adjuvant post-surgery stereotactic radiosurgery was delivered in 5.9% patients. Median follow-up was 27 months (range, 12-252). Symptom improvement was noted in 88.1% of cases, and cranial neuropathies showed improvement in 10.2%. Post-surgical complications occurred in 83 patients (23.5%), mostly dysphagia (7.4%), new cranial neuropathies (6.2%), and cerebrospinal fluid leak (5.9%). A total of 16 patients (4.5%) had tumor recurrence and 7 died (2%), with median overall survival of 2.7 months (range, 0.1-252).
CONCLUSIONS
Microsurgical resection is safe and effective for CVJ schwannomas. Data on SRS efficacy and indications are still lacking, and its role deserves further evaluation.
Topics: Cranial Nerve Diseases; Humans; Neoplasm Recurrence, Local; Neurilemmoma; Radiosurgery
PubMed: 35877244
DOI: 10.3390/curroncol29070384 -
World Journal of Surgical Oncology Mar 2015Cervical vagal schwannoma is a benign, slow-growing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in general population, but... (Review)
Review
Cervical vagal schwannoma is a benign, slow-growing mass, often asymptomatic, with a very low lifetime risk of malignant transformation in general population, but diagnosis is still a challenge. Surgical resection is the treatment of choice even if its close relationship with nerve fibres, from which it arises, threats vagal nerve preservation. We present a case report and a systematic review of literature. All studies on surgical resection of cervical vagal schwannoma have been reviewed. Papers matching the inclusion criteria (topic on surgical removal of cervical vagal schwannoma, English language, full text available) were selected. Fifty-three patients with vagal neck schwannoma submitted to surgery were identified among 22 studies selected. Female/male ratio was 1.5 and median age 44 years. Median diameter was 5 cm (range 2 to 10). Most schwannoma were asymptomatic (68.2%) and received an intracapsular excision (64.9%). Postoperative symptoms were reported in 22.6% of patients. Cervical vagal schwannoma is a benign pathology requiring surgical excision, but frequently postoperative complications can affect patients lifelong, so, surgical indications should be based carefully on the balance between risks and benefits.
Topics: Head and Neck Neoplasms; Humans; Neurilemmoma; Postoperative Complications; Prognosis; Vagus Nerve Diseases
PubMed: 25881101
DOI: 10.1186/s12957-015-0541-6 -
Neurology Nov 2013Neurofibromatosis (NF) is a genetic disease with multiple clinical manifestations that can significantly impact quality of life (QOL). Clinical trials should include... (Review)
Review
OBJECTIVES
Neurofibromatosis (NF) is a genetic disease with multiple clinical manifestations that can significantly impact quality of life (QOL). Clinical trials should include patient-reported outcomes (PROs) as endpoints to assess treatment effects on various aspects of QOL, but there is no consensus on the selection and use of such measures in NF. This article describes the PRO Working Group of the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) Collaboration, its main goals, methods for identifying appropriate PRO measures for NF clinical trials, and recommendations for assessing pain intensity.
METHODS
The REiNS PRO group selected core endpoint domains important to assess in NF. The members developed criteria to rate PRO measures, including patient characteristics, psychometric properties, and feasibility, and utilized a systematic process to evaluate PROs for NF clinical trials. Within the subdomain of pain intensity, the group reviewed the Numerical Rating Scale-11 (NRS-11), the Visual Analogue Scale, and the Faces Pain Scale-Revised using this process.
RESULTS
Based on the review criteria, each of these pain intensity scales is brief, reliable, valid, and widely used. However, the NRS-11 was given the highest rating for use in NF clinical trials due to recommendations from pain experts and other consensus groups, its extensive use in research, strong psychometric data including sensitivity to change, and excellent feasibility in ages ≥ 8 years.
CONCLUSIONS
The systematic review criteria and process are effective for identifying appropriate PRO measures and provide information utilized by the REiNS Collaboration to achieve consensus regarding PROs in NF clinical trials.
Topics: Clinical Trials as Topic; Consensus; Humans; Neurilemmoma; Neurofibromatoses; Pain Measurement; Patient Outcome Assessment; Skin Neoplasms
PubMed: 24249806
DOI: 10.1212/01.wnl.0000435747.02780.bf