ArticleNeuro-oncology2021
Disease course of neurofibromatosis type 2: a 30-year follow-up study of 353 patients seen at a single institution.
Article in Neuro-oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
32 citing papers in PubMed, 48 citations in OpenAlex.
- Multicenter, prospective, phase II study of maintenance bevacizumab for children and adults with NF2-related schwannomatosis and progressive vestibular schwannoma.Neuro-oncology · 2023Trial
- Article
- Management strategies for vestibular schwannomas in patients with NF2-related schwannomatosis: a systematic review.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2026Review
- Development and Initial Validation of the Quality of life Evaluation inmedRxiv : the preprint server for health sciences · 2026Article
- Determinants of the quality of life of patients with NF2-related schwannomatosis and validation of the Dutch NFTI-QOL questionnaire.Familial cancer · 2026Article
- Repurposing anti-retroviral drugs to treat NF2-related tumours: a protocol for a phase 0 trial (RETREAT).BMJ open · 2026Article
- Characterizing the effects of anti-PD1 immunotherapy on ataxia in a vestibular schwannoma mouse model.Journal of neuro-oncology · 2026Article
- Fimepinostat Promotes Apoptosis and Decreases Cytokine Secretion inInternational journal of molecular sciences · 2026Article
- Hearing preservation and restoration surgery in NF2-related schwannomatosis.Familial cancer · 2026Article
- Surgery in NF2-Schwannomatosis.Familial cancer · 2025Review
- BET proteins mediate survival mechanisms in human schwannoma model cells challenged with trametinib but not brigatinib.Oncogene · 2025Article
- Prevalence, natural history and surgical outcome of spinal meningiomas in NF2-related schwannomatosis.Journal of neuro-oncology · 2025Observational
- The clinical spectrum of paediatric NF2- related schwannomatosis.Familial cancer · 2025Review
- History and clinical epidemiology of NF2-related schwannomatosis.Familial cancer · 2025Review
- Multiplatform molecular analyses reveal two molecular subgroups of NF2-related schwannomatosis vestibular schwannomas with distinct tumour microenvironment and therapeutic vulnerabilities.Acta neuropathologica · 2025Article
- Update on Cancer and Central Nervous System Tumor Surveillance in Pediatric NF2-, SMARCB1-, and LZTR1-Related Schwannomatosis.Clinical cancer research : an official journal of the American Association for Cancer Research · 2025Article
- Skull-meninges-brain connectivity and extra-axial brain tumours.Brain communications · 2025Review
- Meningioma: International Consortium on Meningiomas consensus review on scientific advances and treatment paradigms for clinicians, researchers, and patients.Neuro-oncology · 2024Review
- Natural history of hearing and tumor growth in vestibular schwannoma in neurofibromatosis type 2-related schwannomatosis.European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2024Article
- Bilateral vestibular schwannoma with a cooccurring meningioma in a child: a case report and review of literature.Annals of medicine and surgery (2012) · 2024Article
Corrections and comments
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Authors and funding
19 authors at 4 institutions in 2 countries.
Funding
Abstract
backgroundLimited data exist on the disease course of neurofibromatosis type 2 (NF2) to guide clinical trial design.
methodsA prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990 and 2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred, and inheritance type. Interventions for NF2-related tumors were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death.
resultsThree hundred and fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring, 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65, respectively, per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting <16 and >40 years had poorer overall survival than those presenting at 26-39 years (P = .03 and P = .02, respectively) but those presenting between 16 and 39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P = .004).
conclusionUnderstanding disease course improves prognostication, allowing for better-informed decisions about care.
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