Neurofibromatosis type 2
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Bilateral hearing loss, right visual loss and quadriparesis.
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There is a large well defined extra-axial mass in the left cerebellopontine angle, arising from the internal auditory meatus giving the ice-cream cone appearance. It measures 45x40x30 mm in its AP, transverse and longitudinal dimensions. It appears isointense to grey matter on T1, heterogeneously hyperintense on T2/FLAIR and shows avid enhancement post gadolinium administration. No diffusion restriction or blooming artefacts is seen. It exerts mass effect on the pons, left middle cerebellar peduncle, and left cerebellar hemisphere with widening of the adjoining CPA cistern. The fourth ventricle is compressed and displaced to the right with consequent supratentorial hydrocephalus. This appearance is consistent with vestibular schwannoma.
A similar but smaller mass is seen in the right CPA, measures 20x13x10 mm.
Multiple avidly enhancing extra-axial masses of varying sizes are seen along the falx anteriorly, right tentorium cerebellum, left anterior temporal lobe and olfactory groove consistent with multiple meningiomas.
A locally invasive meningioma is also seen in the craniocervical junction encasing the medulla, upper cervical cord, vertebral arteries and the basilar artery. There is infiltration of the adjoining bones and soft tissue more on the left.
A large avidly enhancing mass is seen surrounding the right optic nerve extending into the optic canal and widening it. The optic nerve is seen through the mass (tram-track sign). This appearance is consistent with optic nerve sheath meningioma. There is associated proptosis.
MRI findings of bilateral vestibular schwanmomas, multiple intracranial meningiomas and optic nerve sheath meningioma are consistent with neurofibromatosis type 2.