제2형 다발성 신경섬유종증 1례

Type 2 Neurofibromatosis is one of the neurocutaneous syndromes and it is an autosomal dominant hereditary disease characterized by the development of bilateral vestibular schwannomas.

The diagnostic criteria are met by an individual who has (1) bilateral 8th nerve masses even with appropriate imaging techniques, or (2) a first degree relative with type 2 neurofibromatosis and either a unilateral acoustic neuroma or two of the following : neurofibroma, meningioma, glioma, schwannoma, or juvenile posterior subcapsular lenticular capacity.

Recently we have experienced a case of type 2 neurofibromatosis, manifested with a collateral vestibular schwannoma in a 27-year-old female patient, which was treated with surgical excision. So we report the case with review of literature.