Neurofibromatosis type 2

Synonyms
NF2
ICD-11
LD2D.11

Neurofibromatosis type 2, or NF2 for short, is, along with Neurofibromatosis type 1 and Schwannomatosis, a hereditary tumor syndrome within the neurofibromatosis spectrum.

Diagnostic criteria

Neurofibromatosis type 2 is diagnosed based on the following criteria[^1]:

  • Bilateral Vestibular schwannomas

  • A family history of neurofibromatosis type 2 (first-degree relative) and a unilateral vestibular schwannoma.

  • A family history of neurofibromatosis type 2 and at least two of the following criteria: Meningioma, non-vestibular schwannoma, neurofibroma, glioma, juvenile subcapsular cataract.

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