Cerebellar-pontine angle tumor, rightcMRI axial T1 with contrast mediumCerebellar-pontine angle tumor on the right in a patient with neurofibromatosis type II. These tumors could also be called schwannoma of the vestibular nerve because they originate from it.
This tumor is located in the cerebellopontine angle in the internal auditory meatus, close to the vestibulocochlear and facial nerve.Neurofibromatosis is a genetically-inherited disorder. The severity in affected individuals can vary. Approximately half of cases are due to de novo mutations and no other affected family members are seen. It affects males and females equally. The main feature is the occurrence of multiple benign tumors (schwannomas) in young adults.
The vestibular schwannoma can often lead to ear noises, causing dizziness and vertigo and hearing loss that can lead to deafness. With further growth, these tumors can lead to paralysis of the facial muscles (facial nerve), hoarseness, dysphagia, as well as compression of the brainstem and a hemiplegia.In MRI the tumor shows typical signs of a vestibular schwannoma with predominantly extrameatal parts and a smaller portion located inside the internal acoustic meatus. If the intrameatal portion increases in size it can lead to compression of the nerves located there (cochlear, vestibular, facial nerve) leading to symptomatic nerve lesions.
The surgical treatment of tumors like this is challenging, because of the desired conservation of the adjacant nerves. Especially in larger tumors this often proves impossible.
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