Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Plotkin, Scott R.; Merker, Vanessa L.; Muzikansky, Alona; Barker, Frederick G.; Slattery, William H.

doi:10.1097/mao.0000000000000239

Cited by 62 publications

(39 citation statements)

References 27 publications

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“…The median time to tumor progression was 14 months. A significant number of patients experienced tumor progression and hearing decline over 3 years [Plotkin et al, 2014]. …”

Section: Neurofibromatosismentioning

confidence: 99%

Update from the 2013 international neurofibromatosis conference

Plotkin

Albers

Babovic‐Vuksanovic

et al. 2014

American J of Med Genetics Pt A

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“…The median time to tumor progression was 14 months. A significant number of patients experienced tumor progression and hearing decline over 3 years [Plotkin et al, 2014]. …”

Section: Neurofibromatosismentioning

confidence: 99%

Update from the 2013 international neurofibromatosis conference

Plotkin

Albers

Babovic‐Vuksanovic

et al. 2014

American J of Med Genetics Pt A

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“…Bilateral vestibular schwannomas (VS), which are benign tumors composed of neoplastic Schwann cells that arise from the eighth cranial nerve, are the hallmark of NF2 (2). Standard approaches for treatment of growing VS include surgical removal and radiation therapy (RT).…”

mentioning

confidence: 99%

Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model

Gao

Zhao

Stemmer‐Rachamimov

et al. 2015

Proc. Natl. Acad. Sci. U.S.A.

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Hearing loss is the main limitation of radiation therapy for vestibular schwannoma (VS), and identifying treatment options that minimize hearing loss are urgently needed. Treatment with bevacizumab is associated with tumor control and hearing improvement in neurofibromatosis type 2 (NF2) patients; however, its effect is not durable and its mechanism of action on nerve function is unknown. We modeled the effect anti-VEGF therapy on neurological function in the sciatic nerve model and found that it improves neurological function by alleviating tumor edema, which may further improve results by decreasing muscle atrophy and increasing nerve regeneration. Using a cranial window model, we showed that anti-VEGF treatment may achieve these effects via normalizing the tumor vasculature, improving vessel perfusion, and delivery of oxygenation. It is known that oxygen is a potent radiosensitizer; therefore, we further demonstrated that combining anti-VEGF with radiation therapy can achieve a better tumor control and help lower the radiation dose and, thus, minimize radiation-related neurological toxicity. Our results provide compelling rationale for testing combined therapy in human VS.NF2 schwannoma model | anti-VEGF | neurological function | radiation

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“…Analyses of serial MRI and audiometry studies from this study have been reported in previous publications, (8)(9)(10)(11)) and here we report for the first time Short Form-36 (SF-36) results. Specifically, we aimed to 1) assess patient-reported quality of life in a large cohort of international NF2 patients and 2) explore any associations between HRQoL, demographic characteristics, and objective clinical features.…”

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confidence: 61%

Health-related Quality of Life of Individuals With Neurofibromatosis Type 2

Merker

Bergner

Vranceanu

et al. 2016

Otology &Amp; Neurotology

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Results: At baseline, adults showed significant deficits in all but two subscales of the SF-36 compared to age-and gender-adjusted US population norms. In linear regression models including age, gender, inheritance status, hearing, facial weakness and VS volume, demographic and functional measures showed no relationship to any SF-36 subscale. Larger baseline VS volume was significantly related to reduced physical role performance, reduced mental health, and increased pain (p<0.05). In bivariate analysis, prior VS surgery was not significantly associated with baseline HRQoL; receipt of VS surgery or tumor growth during the observation period was not significantly associated with changes in HRQoL.Conclusion: NF2 patients report reduced HRQoL in physical, social, and mental domains, but this was not significantly related to objective measures of hearing or facial functioning. Larger baseline VS volume negatively impacted patient-reported HRQOL whereas VS surgery or tumor Quality of life in NF2 3 growth did not. Future studies should explore the relationship between tumor volume and HRQoL and psychosocial factors that may moderate this relationship.

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Natural History of Vestibular Schwannoma Growth and Hearing Decline in Newly Diagnosed Neurofibromatosis Type 2 Patients

Cited by 62 publications

References 27 publications

Update from the 2013 international neurofibromatosis conference

Update from the 2013 international neurofibromatosis conference

Anti-VEGF treatment improves neurological function and augments radiation response in NF2 schwannoma model

Health-related Quality of Life of Individuals With Neurofibromatosis Type 2

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