Sagittal MR image of the spine demonstrating a T10-11 schwannoma in a patient with NF2.
Axial MR image demonstrating tumors in a patient with NF2 who presented with the classic bilateral eighth cranial nerve schwannomas that occur in this disease.Neurofibromatosis type 2
-One of the phakomatoses (neuroectodermal disorders with skin and
CNS tumors; other of the more common phakomatoses include tuberous sclerosis, Von-hippel Lindau disease, Sturge-Weber
-Incidence 1:50,000 (much less common than NF-1)
-Autosominal dominant inheritance via chromsome 22 defect
Diagnostic criteria:
-Bilateral vestibular schwanommas are diagnostic
-First degree relative with NF-2 plus unilateral vestibular
schwanomma, meningioma, glioma, scwhanomma, neurofibroma
-Cutaneous manifestations are rare
Radiographic features:
-~100% have cns tumors
-Bilateral vestibular schwanomma is diagnostic
-Other cranial nerve schwanommas
-Meningiomas (can be multiple)
-Non-neoplastic intracranial calcifications (especially
choroid plexus, but can also be cortical)
-Spinal cord schwanommas and ependymomas
