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NF2-Related Schwannomatosis
Built from Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
What’s inside
11 sections · 80 slides
Overview and disease definition
- Definition of NF2-related schwannomatosis
- Tumour spectrum in NF2
- Tumour types in NF2-related schwannomatosis
Genetics and epidemiology
- Inheritance and the NF2 gene
- Merlin and Schwann cell proliferation
- The two-hit requirement
- Prevalence and incidence
- Geographic and ethnic variation
Clinical presentation, diagnosis and natural history
- Typical age and mode of presentation
- Paediatric presentation of NF2
- Bilateral vestibular schwannomas on MRI
- Enhancing cerebellopontine angle schwannoma
- Small intrameatal schwannoma
- Revised diagnostic criteria and nomenclature
- Yield of genetic testing
- Mosaicism in NF2
- Genotype-phenotype correlation
- MRI protocol for detection and surveillance
- Audiological and ophthalmological assessment
- Referral to a high-volume centre
- Natural history and prognosis
- Reduced life expectancy in NF2
Genetic screening and counselling
- Screening at-risk family members
- Reproductive options and informed consent
Treatment of vestibular schwannomas in NF2
- Goals of management
- The four treatment modalities
- Comparing modalities: what the aggregate data show
- Aggregate rates of reported tumor control and hearing preservation in NF2 patients by treatment modality. (Adapted from reference [ 26 ]). Tumor control is defined as no recurrence in the microsurgery group and no change or regression in the other groups. Hearing preservation is defined as no change or an improvement in the Gardner–Robertson hearing grade. Data are shown with the mean follow-up time
Observation
- Growth behaviour of NF2 vestibular schwannomas
- Observation protocol
- Indications to move from observation to treatment
- Early hearing preservation surgery: the controversy
- Features of a favourable tumour for hearing preservation
- Sequencing surgery in bilateral disease
Microsurgical treatment
- Indications for microsurgery
- Gross total versus subtotal resection
- Why NF2 tumours are harder to resect
- Facial nerve outcomes and recurrence
- Hearing preservation rates after microsurgery
- Outcomes in experienced hands
Stereotactic radiotherapy
- Tumour control with stereotactic radiotherapy
- Malignant transformation after radiotherapy
- Evidence for the transformation risk
- Hearing preservation after radiotherapy
- When radiotherapy should be avoided
Systemic medical therapy
- Bevacizumab: drug class and target
- VEGF signalling and angiogenesis inhibition
- Efficacy of bevacizumab in NF2
- Administration and toxicity of bevacizumab
- Rebound growth after stopping bevacizumab
Hearing rehabilitation
- Deafness as the dominant disability in NF2
- Auditory brainstem implantation
- Outcomes with the auditory brainstem implant
- Cochlear implant candidacy in NF2
- Cochlear implant receiver-stimulator and electrode array
- Cochlear implant compared with auditory brainstem implant
- Interpreting the rehabilitation outcome data
- Aggregate hearing rehabilitation results in patients with NF2. Word recognition scores were determined in quiet without lip reading
- Timing of postoperative electrical stimulation testing
Summary and future directions
- Principles of clinical decision-making
- Modality-specific takeaways
- Future directions in NF2 treatment
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Haynes-Glasscock-Shambaugh Surgery of the Ear, Seventh Edition
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