Neurofibromatosis type 2: bilateral vestibular schwannomas

Case contributed by Dr Henry Knipe

Presentation

Hearing loss for investigation.

Patient Data

Age: 40 years
Gender: Female

Enhancing masses completely occupy the internal acoustic canals bilaterally, with extension into the cerebellopontine angles but without mass effect on the brainstem. 

The patient was monitored with serial MRIs for a number of years, with gradual enlargement of the masses. Eventually the patient became symptomatic from brainstem compression, and underwent debulking.

Histology

MICROSCOPIC DESCRIPTION: The sections show a moderately cellular schwannoma. It mainly contains Antoni A areas with fascicles of spindle cells. There is focal palisading with Verocay body formation. A small amount of Antoni B areas with microcystic change is also seen. The tumour cells show focal ancient change with moderate nuclear enlargement and smudged chromatin pattern. Mitoses are inconspicuous. No evidence of malignancy is identified.

DIAGNOSIS: Brain tumour: Schwannoma.

Case Discussion

Bilateral vestibular schwannomas are considered pathognomonic for neurofibromatosis type 2, with other manifestations of this disease including meningiomas and ependymomas. 

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Case Information

rID: 44810
Case created: 5th May 2016
Last edited: 2nd Oct 2016
Inclusion in quiz mode: Included

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