Table 3. Diagnostic criteria for schwannomatosis.

Clinical criteria	Molecular criteria
• At least two nondermal biopsy-proven schwannomas plus no radiographic evidence of bilateral vestibular schwannomas on high-quality MRI or	• Biopsy-proven schwannoma or meningioma plus a germline SMARCB1 mutation or
• One biopsy-proven nondermal schwannoma or intracranial meningioma plus a first-degree relative with schwannomatosis	• At least two biopsy-proven schwannomas or meningiomas harboring a shared SMARCB1 mutation and differing NF2 mutations

Abbreviations: NF2, neurofibromatosis type 2; MRI, magnetic resonance imaging.