Table 2.

NF2 Diagnostic Criteria per 2019 NF Conference Revisions

Finding	Additional Features Required for Diagnosis
Bilateral VS	None
Identical NF2 pathogenic variant in at least 2 anatomically distinct NF2-related tumors	None
One Major criteria (see below)	Additional Major criteria OR two additional Minor criteria (see below)
Major Criteria	Minor Criteria
Unilateral VS	Ependymoma, schwannoma (can count more than one of each type)
First degree relative other than sibling with NF2
Multiple meningiomas	Juvenile subcapsular/cortical cataract, retinal hamartoma, epiretinal membrane if age <40, meningioma (can count each type only once)
NF2 pathogenic variant in an unaffected tissue (e.g. blood)