Neurofibromatosis type 2

Case contributed by Ahmed Abdrabou
Diagnosis certain


Patient with known history of brain tumor and previous VP shunt operation complained of headache and ataxia.

Patient Data

Age: 40 years
Gender: Female

A large right CPA space occupying lesion that displays low signal on T1, high signal on T2 with avid homogeneous enhancement after contrast administration. It shows tongue like extension into the right internal auditory canal and exerts positive mass effect upon the adjacent right middle cerebellar peduncle and right aspect of pons which is compressed and displaced to the left side. There is extensive edema of the right cerebellar hemisphere as well. 

Another similar smaller lesion at the left CPA with the same tongue like extension into the left internal auditory canal.

Both lesions represent vestibular schwannoma. 

Left frontal VP shunt tube is noted with no evidence of hydrocephalic changes.

Case Discussion

Bilateral vestibular schwannoma is pathognomonic of Neurofibromatosis type II. Schwannoma usually involves the inferior division of vestibular nerve and can be differentiated from meningioma by the tongue like extension into the internal auditory canal.

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