Table 1.

Current and Revised Manchester Criteria for Neurofibromatosis Type 2 (NF2)

1. Bilateral VS, diagnosed before age 70

2. First-degree relative family history of NF2 and unilateral VS, diagnosed before age 70

3. First-degree relative family history of NF2 or unilateral VS or 2 of: meningioma, cataract, schwannoma, cerebral calcification (if unilateral VS and > 2 nonintradermal schwannomas, needs negative LZTR1 genetic testing)

4. Multiple meningiomas (2 or more) and 2 of: unilateral VS, cataract, ependymoma, schwannoma, cerebral calcification

5. Constitutional or mosaic pathogenic NF2 gene mutation in blood or identical mutations in 2 distinct tumors.