Editing Neurofibromatosis (section)

== '''NFT-related schwannomatosis''' (formerly called NF2) ==

* Autosomal dominant
* Risks:
** Neurological lesions - schwannomas
** Skin tumours
** Ophthalmological lesions
** Peripheral neuropathy
* Diagnosis - one of the following:
** Bilateral vestibular schwannomas
** At least two anatomically distinct NFT-2 related tumours, with identical pathogenic variant
** Either two major, or one major/two minor criteria:
*** Major
**** Unilateral vestibular schwannoma
**** First-degree relative other than a sibling with NF2-related schwannomatosis
**** Two or more meningiomas
**** NFt pathogenic variant in an unaffected tissue such as blood
*** Minor
**** Ependymoma
**** Schwannoma
**** Juvenile subcapsular or subcortical cataract
**** Retinal hamartoma
**** Epiretinal membrane age <40yo
**** Single meningioma 
* Screening/management
** 3rd-yearly MRI
** Most nerve tumours are managed conservatively