Neurofibromatosis type II

Findings:

Multiple MR images show multiple intracranial enhancing masses, including masses in the left occipital horn and right CP angle cistern/IAC. Postoperative changes of L petrosectomy and fat packing are related to previous left IAC mass resection. A small complex cystic intraaxial mass involves the left cerebellum.

Differential diagnosis:

Nothing reasonable except NF2, confirmed by bilateral IAC masses. Metastases are within possibility, but not typical.

Discussion:

NF2 is AKA MISME, or multiple inherited schwannomas, meningiomas, and ependymomas. Autosomal dominant localized to chromosome 22 with 50% representing new mutations. In contrast to NF1, skin manifestations are minimal.

Diagnostic criteria include bilateral VIII nerve masses, unilateral VIII nerve mass with NF2 in relative, or two of the following:

Neurofibroma, meningioma, glioma, schwannoma, or congenital lens opacity.

In NF2, ependymomas are most common in the spinal cord (80%), most are multiple. Most spinal schwannomas are intradural extramedullary.

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