ArticleJournal of medical genetics2015
Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis Type 2.
Article in Journal of medical genetics, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
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Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 54 citations in OpenAlex.
- EANO guideline on the diagnosis and treatment of vestibular schwannoma.Neuro-oncology · 2020Guideline
- Efficacy and Biomarker Study of Bevacizumab for Hearing Loss Resulting From Neurofibromatosis Type 2-Associated Vestibular Schwannomas.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2016Trial
- USPIO enhanced MR imaging in CNS tumors (UMIC): a study protocol.Nanomedicine (London, England) · 2026Article
- Molecular and genetic insights into vestibular schwannoma.Journal of neuro-oncology · 2026Review
- Molecular pathogenesis of the schwannomatosis genes and genetic testing strategies.Familial cancer · 2025Review
- Multifocal intravestibulocochlear and intracranial vestibular schwannomas: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Multiplatform molecular analyses reveal two molecular subgroups of NF2-related schwannomatosis vestibular schwannomas with distinct tumour microenvironment and therapeutic vulnerabilities.Acta neuropathologica · 2025Article
- A single-cell atlas of Schwannoma across genetic backgrounds and anatomic locations.Genome medicine · 2025Article
- Spontaneous Regression of a Large Vestibular Schwannoma: Is Nonoperative Management Reasonable?Brain tumor research and treatment · 2024Article
- A Novel Multi-Model High Spatial Resolution Method for Analysis of DCE MRI Data: Insights from Vestibular Schwannoma Responses to Antiangiogenic Therapy in Type II Neurofibromatosis.Pharmaceuticals (Basel, Switzerland) · 2023Article
- A mechanistic mathematical model of initiation and malignant transformation in sporadic vestibular schwannoma.British journal of cancer · 2022Article
- Genotype-Phenotype Correlations in Neurofibromatosis and Their Potential Clinical Use.Neurology · 2021Article
- The Neurofibromatoses.Deutsches Arzteblatt international · 2020Review
- An update on the CNS manifestations of neurofibromatosis type 2.Acta neuropathologica · 2020Review
- [18F]fluorothymidine and [18F]fluorodeoxyglucose PET Imaging Demonstrates Uptake and Differentiates Growth in Neurofibromatosis 2 Related Vestibular Schwannoma.Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2019Article
- Familial unilateral vestibular schwannoma is rarely caused by inherited variants in the NF2 gene.The Laryngoscope · 2019Article
- The molecular pathogenesis of schwannomatosis, a paradigm for the co-involvement of multiple tumour suppressor genes in tumorigenesis.Human genetics · 2017Review
- Childhood neurofibromatosis type 2 (NF2) and related disorders: from bench to bedside and biologically targeted therapies.Acta otorhinolaryngologica Italica : organo ufficiale della Societa italiana di otorinolaringologia e chirurgia cervico-facciale · 2016Review
- Comparing the sensitivity of linear and volumetric MRI measurements to detect changes in the size of vestibular schwannomas in patients with neurofibromatosis type 2 on bevacizumab treatment.The British journal of radiology · 2016Observational
- The importance of nerve microenvironment for schwannoma development.Acta neuropathologica · 2016Article
Corrections and comments
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Authors and funding
18 authors at 11 institutions in 2 countries.
Funding
Abstract
backgroundNeurofibromatosis Type 2 (NF2) is a dominantly inherited tumour syndrome with a phenotype which includes bilateral vestibular (eighth cranial nerve) schwannomas. Conventional thinking suggests that these tumours originate at a single point along the superior division of the eighth nerve.
methodsHigh resolution MRI was performed in children genetically proven to have NF2. The superior vestibular nerve (SVN) and inferior vestibular nerve (IVN) were visualised along their course with points of tumour origin calculated as a percentage relative to the length of the nerve.
resultsOut of 41 patients assessed, 7 patients had no identifiable eighth cranial nerve disease. In 16 patients there was complete filling of the internal auditory meatus by a tumour mass such that its specific neural origin could not be determined. In the remaining 18 cases, 86 discrete separate foci of tumour origin on the SVN or IVN could be identified including 23 tumours on the right SVN, 26 tumours on the right IVN, 18 tumours on the left SVN and 19 tumours on the left IVN. DISCUSSION: This study, examining the origins of vestibular schwannomas in NF2, refutes their origin as being from a single site on the transition zone of the superior division of the vestibular nerve. We hypothesise a relationship between the number of tumour foci, tumour biology and aggressiveness of disease. The development of targeted drug therapies in addition to bevacizumab are therefore essential to improve prognosis and quality of life in patients with NF2 given the shortcomings of surgery and radiation treatments when dealing with the multifocality of the disease.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.