Table 5.

Diagnostic criteria of schwannomatosis.

Definite Schwannomatosis

A. Age >30 years and two or more schwannomas (not intradermal), at least one with histologic confirmation with no evidence of vestibular tumor on brain MRI scan and no known NF mutation

B. Vestibular schwannoma (pathologically confirmed) plus first-degree relative who meets the criteria of schwannomatosis

Possible schwannomatosis

A. Age <30 years plus two or more schwannomas (not intradermal), at least one with histologic confirmation with no evidence of vestibular tumor on brain MRI scan and no known NF mutation

B. Age >45 years plus two or more schwannomas (not dermal), at least one with histologic confirmation and no symptoms of 8th nerve dysfunction and NF type 2

C. Evidence of a non-vestibular schwannoma and first-degree relative meeting criteria for definite schwannomatosis

MRI, magnetic resonance imaging; NF, neurofibromatosis.