Table 1 Manchester Criteria for NF2

From: Incidence of mosaicism in 1055 de novo NF2 cases: much higher than previous estimates with high utility of next-generation sequencing

1. Bilateral vestibular schwannomas

OR

2. Family history AND unilateral VS

OR

3. Family history OR unilateral VS AND two of:a meningioma, cataract, glioma, neurofibroma, schwannoma, cerebral calcification (if UVS + ≥2 schwannomas only need negative LZTR1 test)b, OR

 

4. Multiple meningioma (2 or more) AND two of: unilateral VS, cataract, glioma, neurofibroma, schwannoma, cerebral calcification, OR

 

5. Constitutional pathogenic NF2 gene variant in blood or identical in two tumorsb

 
  1. NF2 neurofibromatosis 2, UVS universal vestibular schwannoma, VS vestibular schwannoma.
  2. aIncludes two of any tumor type such as schwannoma.
  3. bRequires molecular analysis.